Twenty-eight patients with a diagnosis of ‘extramammary Paget's disease of the vulva’ were referred to the Radiumhemmet, Karolinska Sjukhuset, Stockholm, during the period 1975–1990. A clinical and histopathologic retrospective review was undertaken. Six patients had associated malignancies (21.4%). The disease was considered primary invasive in three cases, whereas three patients later developed an invasive cancer. Surgery—local resection, hemivulvectomy or vulvectomy—was performed in 24 cases. Twelve patients, in which surgery was supposed to be radical with respect to free margins, had a significantly longer recurrence-free survival than 12 patients in which the surgical margins were dubious.