Primary carcinoma of the male urethra are rare. Among the malignant tumors of the male urethra, squamous cell carcinoma is the most common. Transitional cell carcinoma is very rare, particularly in the distal urethra. We experienced a case of distal urethral transitional cell carcinoma, arising at the fossa navicularis of the penis, which we report here with a review of the literature. A 68-year-old male patient presented with bloody discharge from the prepuce for 1 month. Ultrasonography showed a poorly marginating, heterogeneous mass, invading the glans penis and the corpus spongiosum. The mass encircled the glandular urethra of the penis glans, and obstructed the glandular urethra and the fossa navicularis. A Doppler ultrasonogram revealed hypervascularity in this mass. The mass was isointense to the corpus carvernosum on the T1-weighted images and slightly hypointense to the corpus carvernosum on the T2-weighted images. Contrast-enhanced MR imaging showed a poorly enhancing mass in the glans penis. This mass was confirmed as a transitional cell carcinoma by histologic study and a partial penectomy was performed.