Objective To diffrentiate extramammary Paget’s disease (EPD) clinically and histologically from other skin diseases. Methods Clinical analysis and excisional treatment of 14 patients with EPD were reviewed from 1987 to 1997. Results of 14 patients, 12 involved scrotum and penis, one in the groin and the other one in the syrianal region. All were positive for cytokeratin and negative for S-100 protein. Follow-up showed 3 recurrences who had positive surgical margin biopsy. One died of other diSease. Conclusion Surgery is the first choice for treatment of EPD. Negative margin must he achieved to prevent local recurrence. Objective To diffrentiate extramammary Paget’s disease (EPD) clinically and histologically from other skin diseases. Methods Clinical analysis and excisional treatment of 14 patients with EPD were reviewed from 1987 to 1997. Results of 14 patients, 12 involved scrotum and penis, one in the groin And the other one in the syrianal region. All were positive for cytokeratin and negative for S-100 protein. Follow-up showed 3 recurrences who had positive surgical margin biopsy. One died of other diSease. Conclusion Surgery is the first choice for treatment of EPD. Negative margin must he achieved to prevent local recurrence.