Abstract

Jacques Jean Lhermitte a French neurologist and P. Duclos in 1920 first described a growth in the cerebellum gangliocytoma, as a rare hamartomata’s lesion due to abnormal development of the cerebellum. Since then it has been called by many names Lhermitte-Duclos disease, dysplastic gangliocytoma of the cerebellum, benign hypertrophy of the cerebellar cortex, granular cell hypertrophy and Purkinjeoma. It is a characteristic radiological feature and there has been reported in over 200 cases worldwide in adolescents [1] and old people but a handful of cases are detected and reported in infants. We report a 6 month old male child whose parents reported a history of irritability and typical lion facies with normal mile stones and radiological features of L’hermitte–Duclos disease.