Neuronal ceroid lipofuscinoses (NCL) are inherited neurodegenerative diseases characterized by psychomotor regression, seizures, and visual impairment, due to intracellular accumulation of lipofuscin. CLN5, a subtype manifesting from ages 4 to 17, is particularly rare in non-Finnish populations. Here, we report Japanese first case of CLN5 in an 11-year-old girl with progressive