OBJECTIVE To describe an additional case of desmoid tumor of the penis. The incidence, etiopathogenesis, symptoms, diagnosis and treatment of this condition are discussed. METHODS A 70-year-old male consulted at the emergency services for pain and urinary symptoms. Physical examination showed no regional lymphadenopathy. Patient assessment by ultrasound and CT disclosed a penile lesion. Treatment was by wide local excision. RESULTS Control evaluation at 3, 6 and 12 months showed normal findings and the patient remains asymptomatic. CONCLUSIONS Wide local excision is advocated as the treatment of choice for desmoid tumor of the penis.