Abstract: Objective To study the clinical characteristics,treatment modalities and prevention measures of congenital megacolon accompanying enterocolitis（EC）. Methods Clinical data about 82 congenital megacolon patients（including 21 were complicated with EC） admitted to our hospital from April 1993 to April 2012 were retrospectively analyzed. Results Of the 21 patients with congenital megacolon accompanying EC,18 were complicated with EC before operation and 3 were complicated with EC after operation.Of the 8 patients who underwent intestinal fistulation,7 were cured and 1 died.Of the 13 patients who did not undergo fistulation,11 were cured and 2 died after conservative treatment.No significant difference was found in survival rate between the patients who underwent fistulation and those who did not underwent fistulation（P＞0.05）. Conclusion Enemata and intestinal fistulation are the two effective treatment modalities for EC.Occurrence of EC before operation and operation time interval are the two important factors for the occurrence of EC after operation.