Canavan disease (CD) is a rare monogenic neurodevelopmental disorder caused by autosomal recessive loss-of-function mutations in the ASPA gene encoding aspartoacylase (ASPA).1 ASPA is expressed by oligodendrocytes in the central nervous system (CNS) where it hydrolyzes N-acetylaspartate (NAA), its only known substrate, into acetate and aspartate.1,2 No other enzyme is known to hydrolyze NAA and the high levels of NAA observed in CD are considered pathognomonic.1 Here, ASPA is linked to neurodevelopmental disorder.