We report two cases of total colonic aganglionosis in which the ileocecal valve and right colon were preserved. The aganglionic ileal segment is removed leaving behind only a very short prececal remnant, which is anastomosed end-to-back to the remaining ileum. An ileal loop (of approximately 20 cm in length) is isolated and interposed between the right colon and rectum. The operation can be performed as a staged procedure. The advantage of preserving the ileocecal valve with respect to physiology is discussed.