Priapism associated with Niemann-Pick disease in a 15-year-old boy

A 15-year-old boy presented with priapism of 46 h duration. There was no known cause of priapism detected in him. During evaluation, biopsy of the lymph node and bone marrow aspiration detected patient to be having Niemann-Pick disease. He was managed with cavernous aspiration, saline irrigation, and intracavernosal phenylephrine injection. Although priapism is associated with many known diseases, about half of the cases are of idiopathic origin. We are reporting the first case of priapism associated with Niemann-Pick disease in the literature.