There are subsets of chronic constipation patients showing features of colonic pseudo-obstruction (CPO) with distinct transitional zone (TZ). We intended to analyze the clinicopathologic characteristics and surgical outcomes of these patients. Twenty-five consecutive patients who underwent surgery for constipation over the 9-year period were analyzed. TZ (+) group was defined as patients showing symptoms or signs of large bowel obstruction with dilated proximal and collapsed distal colon around the TZ at the time of operation, but without any evidence of mechanical causes of obstruction. Nineteen (76%) patients had features of CPO with TZ. All TZs were located in the left colon. Pathologically, segmental hypoganglionosis was identified at the TZ in all TZ (+) patients. On the other hand, pathologic diagnosis was intestinal neuronal dysplasia type B in the remaining six (24%) patients having a uniform colon diameter without demonstrable dilatations (TZ (-) group). Among TZ (+) patients, 17 (90%) underwent total colectomy with ileorectal anastomosis and two (10%) underwent enterostomy. Long-term follow-up (median 56 months) showed no recurrence of constipation in this group of patients. All six TZ (-) patients underwent total colectomy with ileorectal anastomosis and two (33%) of them had persistent symptoms of constipation on long-term follow-up (median 60 months). In a subset of adult constipation patients presenting with features of CPO with TZ, segmental hypoganglionosis was the final pathologic diagnosis. Constipation patients with features of CPO with distinct TZ in the left colon are expected to benefit from surgical intervention.