Primitive Neuroectodermal Tumor of the Vulva in an Adolescent Girl

Extraskeletal primitive neuroectodermal tumor (PNET) is rare and that affecting the female genital tract is extremely rare. We report the case of a 17-year-old girl who presented with swelling of the clitorus. She underwent excision and histopathology was PNET. She received chemotherapy and radiation and achieved complete remission. However, she relapsed locally and systemically soon, progressed and died.