Canavan disease is an autosomal recessive degenerative disorder that causes progressive damage to nerve cells in the brain. Canavan disease is one of the most common degenerative cerebral diseases of infancy. This disease is one of a group of genetic disorders called leukodystrophies.

Leukodystrophies are characterized by degeneration of myelin in the phospholipid layer insulating the axon of a neuron. The gene associated with the disorder is located on human chromosome 17.