Here, we describe a 37 year old patient in a clinically early stage of AD-ANCL harboring a p.L115R mutation in DNAJC5/CSPα. This patient was a member of a multigenerational family with AD-ANCL, which has been clinically and pathologically described by Josephson et al. [5]. Here, DNAJC5 is linked to adult neuronal ceroid lipofuscinosis.