Introduction: Scrotal lymphedema is a rare condition, with significant psychological and functional disability. To date, association with giant congenital melanocytic nevus has not been reported. Case report: We report a case of a 15-year-old male with a giant congenital nevus associated with giant scrotal lymphedema. Surgical debulking with penoscrotoplasty achieved satisfactory functional and esthetic results. Conclusions: Early diagnosis and surgical intervention should be advocated for congenital causes of large scrotal swelling.
