An 11-year-old girl with juvenile neuronal ceroid lipofuscinosis devel
oped acute pancreatitis secondary to valproic acid treatment, which re
solved after discontinuation of the medication and conservative treatm
ent. To my knowledge, this is the first reported case of neuronal cero
id lipofuscinosis with valproate-associated pancreatitis. Children wit
h neuronal ceroid lipofuscinosis may be susceptible to developing this
complication.