Urethral carcinoma is a rare cancer of the urinary tract. Although most cases are of squamous cell carcinomas, there are several reports in the literature about transitional cell carcinoma and adenocarcinomas whose origin remains controversial. While the diagnosis of this condition is essentially clinical, magnetic resonance imaging (MRI) is the examination of choice for its staging, and a pathological confirmation is still necessary. There is no consensus on the treatment of these tumors because of its low incidence. The following therapeutic options are currently available: surgical resection (often for small and distal urethral tumors); radiotherapy and chemotherapy, for larger tumors, seeking a functional preservation of the penis, bladder and/or vagina; or a combination of these therapies thereof in case of more extensive tumors. The classic treatment involves surgery aiming loco-regional disease control, and continent urinary derivation provides satisfactory functional results in cases of radical urethrectomy. Much still needs to be learned about urethral cancer, and molecular diagnostics tools and therapeutic targets are promissory. The key to better understanding urethral cancer is the pooling of data from a wide range of sources including international consortia.