A 67-year-old man noted a 4×6cm sized, erythematous, erosive, oozing patch on the penile shaft, which resisted conservative therapy for three years. Histopathologic examination revealed typical findings of extramammary Paget's disease. Interestingly, nests of Paget's cells in some hair follicular structures were shown in the middermis. The lesion was excised by microscopically controlled excision, using the fresh-tissue technique. There were histologically involved areas extending to quite a distance beyond the clinically normal appearing skin. Mohs micrographic surgery might offer the unique advantage of effective surgical treatment with microscopically controlled tumor free borders in the extramammary Paget's disease.