Neuronal ceroid lipofuscinosis is one of the heredodegenerative diseases fo
r which clinical and neuropathologic findings are well documented. We prese
nt a patient with late infantile neuronal ceroid lipofuscinosis with true p
recocious puberty; to our knowledge, this association has not been reported
before. The association could be due to an underlying disturbance of hypot
halamic-pituitary gonadal function, or to coincidence.