Lymph node metastasis from carcinoma in situ of the penis is rare. We report a case of carcinoma in situ of the penis that was initially managed with circumcision and topical 5-fluorouracil. Subsequently inguinal and para-aortic lymph node metastasis developed, which was treated successfully with systemic chemotherapy and right inguinal lymph node dissection. The patient is free of disease 7 years after initial diagnosis and 3 years after the diagnosis of nodal metastasis.