Genital Neurofibromatosis Presenting as Painful Clitoromegaly

Neurofibromatosis type 1 (NF1) is a neurocutaneous disorder caused by a mutation on chromosome 17 of the gene for neurofibromin. Patients with this condition are prone to develop benign and malignant tumors of the central and peripheral nervous systems. Genitourinary involvement in children with NF1 is uncommon and primarily affects the bladder. External genital manifestations of NF1 are rare with sporadic reports of children presenting with enlargement of their penis, clitoris, or labia due to a neurofibroma. We present a case of painful clitoromegaly due to a plexiform neurofibroma in a child. (C) 2019 Elsevier Inc.