Biallelic CLN3 (OMIM *607042) variants have been associated with not only JNCL but also early‐ or late‐onset isolated retinal dystrophy without neurological signs (Chen et al., 2019; Ku et al., 2017; Lerner et al., 1995; Munroe et al., 1997; Wang et al., 2014). Here, CLN3 is linked to juvenile neuronal ceroid lipofuscinosis.