Hirschsprung disease (HD) is characterized by aganglionosis, which mainly Occurs in the rectum and distal sigmoid colon. Typical HD is seldom diagnosed in adulthood, and segmental involvement is very rare. A 37-year-old man suffered from refractory constipation for 20 years. He could only defecate once a week and frequently needed an enema for defecation. A barium enema showed an annular stenotic segment of the rectosigmoid colon of 8 cm in length, which started 10 cm above anal circulation and showed dilated colon above the stenotic segment. The distal rectum was normal. The narrowed segment of the rectum was resected. At one-year follow up, the patient had normal defecation without laxatives.