Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy, usually on the genitalia, that almost always extends beyond clinically apparent margins. Recurrences after standard methods of surgical excision are notoriously frequent; effective treatment with Mohs micrographic surgery was first reported in 1979. It has since been suggested this malignancy may be multifocal, and reports of recurrences after resection with micrographic surgery have appeared. The authors report six cases treated with Mohs surgery, two of which recurred. They also present data on 42 additional cases obtained from a written survey of members of the American College of Mohs Micrographic Surgery and comparison cases selected from the literature. The recurrence rate after micrographic surgery appears to be at least as low as that after conventional surgical excision with vertical frozen section or paraffin section margin control. Mohs micrographic surgery allows for maximal tissue sparing of critical anatomic structures and is performed under local anesthesia as an outpatient; because of this, it may be superior to conventional surgical excision. A scheme for management of this malignancy is presented. Surgeons should be aware radical excision is not needed for most cases of extramammary Paget's disease and very long-term patient follow-up is required.