ABSTRACT
The objective is to describe an uncommon case of Hirschsprung's disease (HD), also called congenital megacolon. The lack of neuronal ganglion cells from the submucosal and muscular nerve plexuses throughout a segment of varied length characterizes HD. Approx 94% of HD cases are diagnosed below the age of 5 years. A 3 years old male patient was admitted to the male surgery ward under a pediatric surgeon with the chief complaints of chronic constipation since birth with a history of delayed passage of meconium. The patient was diagnosed with a case of HD at the age of 1 year for which he was operated on colostomy was performed with multiple biopsies from narrowed rectosigmoid, from the transitional zone, and from colostomy stoma which confirmed the decision to make colostomy at the ganglionated segment of the colon based on the clinical picture during surgery. Biopsy report of narrowed rectosigmoid segment showed the absence of ganglion cells, while colostomy stoma and dilated proximal colon showed the presence of ganglion cells. The patient underwent a surgical procedure named pull-through surgery for HD, a martin's modification of the Duhamel operation.