A one and half years male child presented with constipation with severe colicky abdominal pain, bilious vomiting & abdominal distension. He had history of recurrent bouts of constipation followed by gastroenteritis since birth for which he had taken symptomatic treatment & sometimes remained symptom free but he had no other significant history or associated condition. In laboratory investigations, barium enema study of large gut result simulates to Hirschsprung's disease but suction rectal biopsy revealed normal rectal tissue texture. So, consideration of diagnostic tools along with patient's general condition decision was taken for diagnostic laparotomy & peroperatively the child was diagnosed as a case of intestinal obstruction due to congenital colonic stenosis in the descending colon. After resection of stenotic segment and end to end anastomosis, histopathologycal examination of resected stenosed colon was done & it was finally proved as congenital stenosis in the descending colon. The post operative period of the patient was uneventful and he was discharged on 7th postoperative day & followed up upto 6 months. He had been found alright without any complain. Here we tried to high light that the congenital colonic stenosis as a rare, but might be a possible cause of partial/complete intestinal obstruction from newborn to older children in any part of the colon & that should kept in mind for avoiding diagnostic dilemma & proper management of patient.