Pseudoepitheliomatous hyperplasia, keratotic and micaceous balanitis(PEKMB) Are extremely rare, and can be mistaken for squamous cell carcinoma of the penis. A case of PEKMB in a 62-year old patient is presented, and the lesion has been treated successfully with circumcision and topical 5-fluorouracil cream, with no evidence of recurrence at 1-year follow-up. We review previously reported cases, and discuss the diagnosis and treatment of this entity.