Extramammary Paget's disease presenting on the face.

Background: Extramammary Paget's disease is a rare neoplasm primarily affecting apocrine gland bearing skin. Although primarily affecting the anogenital area, the tumor also rarely appears in nonapocrine bearing skin and is referred as ectopic extramammary Paget's disease.

Objective: To our knowledge, we present only the second case of ectopic extramammary Paget's disease appearing on the face.

Methods: Using Mohs micrographic surgery, a rare case of ectopic extramammary Paget's disease on the face was treated in three stages.

Results: At 5 months there was no evidence of recurrence.

Conclusion: Ectopic extramammary Paget's disease is a rare disease that can be effectively treated with Mohs micrographic surgery.