Canavan disease is an inherited disorder that causes progressive damage to the nerve cells of the brain due to the deficiency of the enzyme aspartoacylase. Infantile Canavan disease is the more severe form, where symptoms occur at 3 to 5 months of age and include loss of motor skills, feeding and swallowing difficulties, abnormal muscle tone, and a poorly controlled and abnormally large head. Deafness, blindness, and paralysis can also occur. Life expectancy is variable, from within the first few years of life to their teens, depending on severity of symptoms manifested and medical management and care of symptoms. Children with the milder juvenile form may appear quite normal with only mild speech or developmental delay. They can survive into their teens and beyond. Currently, there is no standard treatment to lessen disease progression, but medical surveillance and care may help to improve some symptoms and overall condition of life. Canavan disease is caused by pathogenic variants in the ASPA gene.