We report a case of transitional cell carcinoma of the fossa navicularis in an elderly white male. The patient presented in urinary retention, with a large exophytic mass at the external urethral meatus. Both the pathogenesis and natural history of this highly unusual tumor are unclear. Treatment depends on grade and stage of disease. Transurethral resection and fulguration have been successfully used to treat superficial lesions. Segmental resection and partial penectomy, with or without inguinal lymph node dissection, form the mainstay of treatment for invasive disease. The role of chemotherapy for advanced disease is unknown.