Background: Although extramammary Paget’s disease (EMPD) is a rare skin neoplasm and of which has an associated internal malignancy is rarer, it is known that approximately 15-20% of EMPD cases are associated with an underlying internal malignancy. Objectives: The aim of this study is to identify the real incidence of concomitant malignancies in EMPD patients in Korea and to think about the proper approach to the malignancies. Methods: The 85 patients confirmed with EMPD from June 2005 to June 2015 were included in this study. Clinical records of the patients were reviewed retrospectively. We also reviewed literatures including information about EMPD and concomitant malignancy in Korean patients. Results: In total of 362 patients, mean age was 67.1 years and a ratio of male to female was 8:2. The most common predilection site was penoscrotal area(90.0%). The concomitant malignancies with EMPD were shown in 49 cases(13.5%). The confirmed associated malignancy by guideline was 31(8.6%) cases. Conclusion: The real incidence of associated malignancy in EMPD patients in Korea was lower than known before, but they showed diverse kinds of malignancies. Therefore, we recommend being aware of the possibility of concomitant malignancy in EMPD patients, and the regular health medical examination depending on their ages rather than the various kinds of routine evaluations after diagnosis of EMPD.