Rectal ectasia may be associated with anorectal anomalies, If not recognized at the time of surgical reconstruction it may lead to megarectosigmoid, resulting in severe constipation and overflow incontinence postoperatively, The authors treated four patients presenting with this condition, One patient born with a low anorectal anomaly and two with high anorectal anomalies experienced intractable constipation caused by megarectum despite otherwise adequate primary reconstructive procedures, A fourth patient had rectal stenosis in association with megarectosigmoid, The ectatic megarectum had to be resected in all the patients to achieve normal bowel actions. The authors feel that resection or tailoring of the ectatic segment should be an integral part of the primary reconstructive procedure. Copyright (C) 1997 by W.B. Saunders Company.