Background Various methods have been described to close large meningomyelocele defects, but no technique has been proven superior to others. This study presents cases of meningomyelocele defect closure with a keystone-design perforator island flap.Methods A retrospective study was performed on 14 patients with meningomyelocele defects closed using various types of keystone flaps.Results The median age of the patients at surgery was 10.5 days (range, 1–369 days) and the average defect size was 22.5 cm2 (range, 7.1–55.0 cm2). The average operative time for defect closure was 89.6 minutes (range, 45–120 minutes). Type IV bilateral keystone flaps were used for four defects, type IV unilateral flaps for six defects, type IIA flaps for two defects, and type III flaps for two defects.Conclusions All the defects healed completely with no major complications. The keystone-design perforator island flap is a reliable, easy, and fast technique to close large meningomyelocele defects.