Background There has been little research on extramammary Paget's disease (EMPD) conducted in Asia. Methods The aim of this multicenter study was to investigate the clinicopathological features, biological behavior, and treatment of EMPD in 194 Korean patients over a five-year period. Results As expected, this disease exhibited a male dominance (3.9:1) pattern. To distinguish the true association of malignancy with EMPD from coincidental occurrence of malignancy, we modified Chanda's guidelines. This analysis determined that there was an accompanying malignancy in 26 patients (14.4%), which included stomach (six cases), rectum (three cases), and colon (three cases) malignancies. With regard to treatment, 112 patients (58%) underwent conventional excisions, and 22 (11.4%) had Mohs surgery. Follow-up data for periods of 3-180months were available for 147 patients; these data indicated that 42 patients (28.6%) experienced local recurrences. Patients who underwent Mohs micrographic surgery experienced a lower rate of recurrence (12.5%) than patients who underwent conventional excision (33.3%). Conclusions In summary, this Korean population-based study demonstrated that the clinical features of EMPD, such as gender predominance, are consistent with reports from other Asian population-based studies. However, this study additionally demonstrated that the rate of association with malignancy in the Korean population (14.4%) was higher than that observed in other Asian studies.