diff --git a/homeassistant/components/zwave_js/__init__.py b/homeassistant/components/zwave_js/__init__.py
index a4eb466fe87..82e79b83659 100644
--- a/homeassistant/components/zwave_js/__init__.py
+++ b/homeassistant/components/zwave_js/__init__.py
@@ -102,6 +102,20 @@ async def async_setup_entry(hass: HomeAssistant, entry: ConfigEntry) -> bool:
         # update entity availability
         async_dispatcher_send(hass, f"{DOMAIN}_{entry.entry_id}_connection_state")
 
+        # Check for nodes that no longer exist and remove them
+        stored_devices = device_registry.async_entries_for_config_entry(
+            dev_reg, entry.entry_id
+        )
+        known_devices = [
+            dev_reg.async_get_device({get_device_id(client, node)})
+            for node in client.driver.controller.nodes.values()
+        ]
+
+        # Devices that are in the device registry that are not known by the controller can be removed
+        for device in stored_devices:
+            if device not in known_devices:
+                dev_reg.async_remove_device(device.id)
+
     @callback
     def async_on_node_ready(node: ZwaveNode) -> None:
         """Handle node ready event."""
diff --git a/tests/components/zwave_js/conftest.py b/tests/components/zwave_js/conftest.py
index b6cbc911b6a..0e0ebdee3c6 100644
--- a/tests/components/zwave_js/conftest.py
+++ b/tests/components/zwave_js/conftest.py
@@ -308,3 +308,15 @@ def in_wall_smart_fan_control_fixture(client, in_wall_smart_fan_control_state):
     node = Node(client, in_wall_smart_fan_control_state)
     client.driver.controller.nodes[node.node_id] = node
     return node
+
+
+@pytest.fixture(name="multiple_devices")
+def multiple_devices_fixture(
+    client, climate_radio_thermostat_ct100_plus_state, lock_schlage_be469_state
+):
+    """Mock a client with multiple devices."""
+    node = Node(client, climate_radio_thermostat_ct100_plus_state)
+    client.driver.controller.nodes[node.node_id] = node
+    node = Node(client, lock_schlage_be469_state)
+    client.driver.controller.nodes[node.node_id] = node
+    return client.driver.controller.nodes
diff --git a/tests/components/zwave_js/test_init.py b/tests/components/zwave_js/test_init.py
index b17945d05c6..86fbf27ab4f 100644
--- a/tests/components/zwave_js/test_init.py
+++ b/tests/components/zwave_js/test_init.py
@@ -7,6 +7,7 @@ from zwave_js_server.model.node import Node
 
 from homeassistant.components.hassio.handler import HassioAPIError
 from homeassistant.components.zwave_js.const import DOMAIN
+from homeassistant.components.zwave_js.entity import get_device_id
 from homeassistant.config_entries import (
     CONN_CLASS_LOCAL_PUSH,
     ENTRY_STATE_LOADED,
@@ -14,6 +15,7 @@ from homeassistant.config_entries import (
     ENTRY_STATE_SETUP_RETRY,
 )
 from homeassistant.const import STATE_UNAVAILABLE
+from homeassistant.helpers import device_registry, entity_registry
 
 from .common import AIR_TEMPERATURE_SENSOR
 
@@ -290,3 +292,42 @@ async def test_remove_entry(hass, stop_addon, uninstall_addon, caplog):
     assert entry.state == ENTRY_STATE_NOT_LOADED
     assert len(hass.config_entries.async_entries(DOMAIN)) == 0
     assert "Failed to uninstall the Z-Wave JS add-on" in caplog.text
+
+
+async def test_removed_device(hass, client, multiple_devices, integration):
+    """Test that the device registry gets updated when a device gets removed."""
+    nodes = multiple_devices
+
+    # Verify how many nodes are available
+    assert len(client.driver.controller.nodes) == 2
+
+    # Make sure there are the same number of devices
+    dev_reg = await device_registry.async_get_registry(hass)
+    device_entries = device_registry.async_entries_for_config_entry(
+        dev_reg, integration.entry_id
+    )
+    assert len(device_entries) == 2
+
+    # Check how many entities there are
+    ent_reg = await entity_registry.async_get_registry(hass)
+    entity_entries = entity_registry.async_entries_for_config_entry(
+        ent_reg, integration.entry_id
+    )
+    assert len(entity_entries) == 18
+
+    # Remove a node and reload the entry
+    old_node = nodes.pop(13)
+    await hass.config_entries.async_reload(integration.entry_id)
+    await hass.async_block_till_done()
+
+    # Assert that the node and all of it's entities were removed from the device and
+    # entity registry
+    device_entries = device_registry.async_entries_for_config_entry(
+        dev_reg, integration.entry_id
+    )
+    assert len(device_entries) == 1
+    entity_entries = entity_registry.async_entries_for_config_entry(
+        ent_reg, integration.entry_id
+    )
+    assert len(entity_entries) == 9
+    assert dev_reg.async_get_device({get_device_id(client, old_node)}) is None