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Purpose To evaluate the effect of repeated debulking surgery for high-grade pseudomyxoma peritonei (PMP) originating from the appendix. Methods Between January 1998 and December 2014, fifty patients, who underwent debulking surgery for high-grade PMP originating from the appendix, were obtained from a prospectively collected database and retrospectively analyzed. Two groups according to the number of operations were divided and analyzed. Results A total of 118 operations were performed. Thirty-one patients received more than two operations. The median interval between operations was 18.2 months (range, 2–170 months). Complications developed after 26 operations (22.0%), including ileus (n=10), intra-ab-dominal fluid collection (n=7), surgical site infection (n=5), and others. There were two mortalities within 30 days after operation. Between two groups of patients who received one operation only and patients who received more than two operations, transfusion, diversion operation, and postoperative complication rate showed statistically significant differences. Two groups of patients had no differences in overall survival rates. Conclusion Our results indicate that the number of operations does not affect the survival rate of high-grade appendiceal PMP, in which repeated debulking surgery is vital to relieve symptoms of the tumor burden.