Hirschsprung's disease in the adult is a rare and frequently misdiagnosed cause of long-standing refractory constipation. We report a case of Hirschsprung's disease in a 23-year-old man and review the literature. The patient had a history of chronic constipation that required daily enemas, since early infancy, but he had remained in good health until intestinal obstruction developed. As a subemergency operation, right transverse colostomy was performed, to relieve the constipation. Histological examination, by a biopsy, showed absence of ganglion cells in the myenteric plexus in the rectum. One year later, Ikeda's modification of the Duhamel procedure was successfully performed as definitive surgery. The postoperative course was uneventful, and complete resolution of the symptoms without complications has been confirmed by a 22-year follow-up. A review of 229 cases of adult Hirschsprung's disease in the literature suggested that the Duhamel procedure is the operation of choice because of the lower postoperative morbidity rate and better functional outcome.