Objective: To present a new case of a primary clear cell adenocarcinoma of the urethra and its surgical management.
Material and methods: We describe the clinical, diagnosis, treatment and development of this kind of tumor. Review of the literature.
Conclusions: It is an unusual type of cancer associated with poor prognosis. Currently the construction of a continent urinary diversion using the Mitrofanoff principle has many indications as our case. Laparoscopic radical cystectomy can be done by experienced groups without adding much more technical difficulties; there are no long-term oncological outcome data but we believe in some functional advantages.