We conducted a retrospective review of the medical records of 18 patients diagnosed at the University of Colorado with perineal melanoma. Clinical characteristics including Clark's level, recurrence, and survival were studied. Ten patients (55.6%) had vulvar melanoma, 2 patients (11.1%) had vaginal disease, and the remaining 6 patients (33.3%) had primary rectal melanoma. All patients were treated surgically at the time of diagnosis. At a median follow-up of 32.7 months, 15 patients had evidence of recurrence. Nine of these patients are dead and six are alive with disease. The average time to recurrence was 14 months and the most common site was the regional lymph nodes in 10 of the 14 patients (71.4%). This study confirms the poor prognosis in perineal melanoma and the only effective treatment to date is early diagnosis and complete resection.