BACKGROUND: Extramammary Paget's disease (EMPD) is a rare clinical entity and can be associated with other malignancies. We analyzed our experience for prognosis and impact of therapy on outcomes. STUDY DESIGN: We conducted a retrospective review of all patients (n = 33) with EMPD treated at a tertiary care center from 1971 to 1998. Pathologic features of EMPD, concurrent secondary malignancies, and the effect of operations on recurrence were analyzed. Overall survival was compared with that of the general population, RESULTS: Male-to-female ratio was 4:29, and median age was 70 years. Median followup was 68 months, and no patient died from EMPD. The lesion was predominantly found on the vulva (76%). Patch-like, nonconfluent growth was present in 45% of patients, and no patient had pathologic lymph nodes. The most common signs and symptoms were irritation or pruritus (73%) and rash (61%). The presence of patches, invasive tumor growth, or a second malignancy were significantly associated with a higher recurrence rate. The type of operation, either local excision or hemivulvectomy, was not related to the time to recurrence. Complete gross resection was achieved in 94% of cases. Fifty-six percent of patients had microscopically positive margin and this correlated with a significantly higher recurrence rate (p = 0.002). The tumor recurred clinically in 14 of 33 patients (42%) after a median of 152 months (range 5 to 209 months). In those patients, between one and six reexcisions were performed. In 14 of 33 patients with EMPD (42%), 16 concurrent secondary malignancies were found. Overall survival rates for EMPD patients were similar to those of the general population. CONCLUSIONS: EMPD is an infrequently diagnosed disease that is preferably managed with complete local excision and reexcisions if needed. A thorough search for frequently occurring secondary malignancies might be beneficial to provide the best outcomes for these patients.