We recently reported a novel canine lysosomal storage disease (LSD) with similarities to the neuronal ceroid lipidoses in two Dalmatian littermates with a homozygous nullifying single-base deletion and reading-frame shift in CNP, the gene that encodes a protein with 2′,3′-cyclic-nucleotide 3′-phosphodiesterase enzyme activity (CNPase) and a structural role in myelin [1]. Here, CNP is linked to lysosomal storage disease.