Neurofibromatosis is a common inherited autosomal dominant disease, but genitourinary neurofibroma is rare. The unpredictable nature of neurofibromas has a serious impact on the quality of life of patients, and their management is challenging for clinicians. We present a 9-year-old girl with plexiform neurofibroma of genitourinary system associated with pulmonary hypertension, masquerading as sacrococcygeal teratoma. Intraoperative finding and histological examination of the resected tumor confirmed the diagnosis.