Congenital short colon (CSC) is a condition in which the colon is replaced, wholly or partially, by a dilated pouch together with anorectal malformation and a colourinary fistula. Most of the reported series have been from northern India where this malformation is frequent. This paper details the management of 108 patients with CSC over a period of 23 years. The patients were classified into two types: (1) Partial short colon, where a segment of normal colon is present between the ileum and the sac. These patients could be treated by excision of the pouch and pull-through of the segment of normal colon during either single- or two-stage surgery. (2) Complete short colon, where the ileum opened directly into the sac, and formation of a tube from this sac (coloplasty) was required in one or more stages to provide a length of normally functioning colon. Review of the results showed staged management to be superior. The follow-up has ranged between 3 and 7 years, with satisfactory long-term results in both groups of patients. We have evolved a protocol for the management of CSC that has improved the prognosis and quality of life of these patients.