Primary vaginal melanoma is a very rare gynecological malignant tumor (less than 150 reported cases to-date). Prognosis is poor in spite of treatment. Due to the fact that only small groups of patients have been compared, conservative treatment has usually been recommended. In recent times, radical pelvic surgery has appeared to improve the chances of survival. We present an unusual case of primitive melanoma of the upper-third of the vagina with urethral and urinary bladder infiltration in a 47-year-old woman. Treatment consisted of preliminary pelvic bilateral lymphadenectomy, anterior exenteration, and urinary bladder reconstruction according to the Bricker technique. Four months after surgical treatment, liver metastases were found. Chemotherapy was initiated consisting of 8 cycles every 21 days of fotemustine 100 mg/m2 (day 1) and dacarbazine (DTIC) 250 mg/m2 (days 2-5). Interferon alpha-2-b, 3 MU thrice weekly, for the whole period of chemotherapy, was also administered. The patient is in partial remission one year after surgical treatment.