Neurilemoma or Schwannoma is a rare benign tumor which may affect any nerve of the body. Genitourinary involvement is rare. Herein we describe a case of neurilemoma of the perineum. To our knowledge, only one case involving this site has been previously described in the literature.We report on a patient with an asymptomatic, solitary perineal tumor. The presumptive diagnosis of a benign tumor was made on the basis of the ultrasound and CT findings. Pathological analysis disclosed the typical Verocay bodies of a neurilemoma.The treatment of choice is by simple surgical excision. Tumor recurrence is rare.