Ileocecal valve atresia is the most uncommon yet remarkable form of the atresia found within the gastrointestinal system. We report a case on this rare entity with few cases documented in the literature to date. In our case, a one-day-old full-term male infant who developed the signs of intestinal obstruction was eventually taken for emergency laparotomy. The atretic area found intraoperatively was removed followed by the creation of an anastomosis. The patient recovered well postoperatively and continues regular pediatric follow-ups.