Seventy-two patients with complete short colon (CSC) were treated at the authors' institution between 1971 and 1994. Initially, one-stage coloplasty with abdominoperineal pull-through was performed in 10 neonates, with a 50% survival rate. The subsequent 62 patients had staged management in which a window colostomy was performed at the time of presentation. Of the 50 survivors in this group, 20 had coloplasty with a abdominoperineal or sacroabdominoperineal pull-through by June 1994. A protective ileostomy was done at the time of coloplasty in 10 patients, and was closed 6 to 12 weeks later. Two of the 20 patients who had staged reconstruction died because of complications; the others recovered well. The overall survival rate after coloplasty was 76.7%. Eighteen of the 23 survivors have had follow-up for up to 8 years. They have shown normal growth and good continence and have been free of significant long-term complications. The other 30 patients are awaiting definitive surgery. A staged management plan is advocated for complete short colon because it provides consistent results and good quality of life.