Introduction and importance: Enteric duplication cyst (EDC) is a rare congenital anomaly with incidence of 1 in 4500 live birth. Meckel’s Diverticulum (MD) is the most common congenital anomaly of gastrointestinal (GI) tract. The coexistence of EDC and MD in same patient is extremely rare. Presentation of EDC depends on it’s location, may present with dysphagia, respiratory distress, abdominal pain, abdominal distension and intestinal obstruction. However, MD remains asymptomatic in most of the cases. Case presentation: We present a case of 11month boy, presenting with fever, vomiting, and abdominal distension for four days and decreased frequency of defecation with fowl smelling stool, not mixed with blood. All the systemic examination findings were regular except soft and generalized distension of abdomen. CECT scan of abdomen showed features suggestive of EDC versus mesenteric cyst. The patient underwent exploratory laparotomy with ileocecal resection, ileocolic anastomosis and appendectomy. Meckel’s diverticulum was discovered incidentally at distal ileum, and was left intact. The patient was discharged in stable state. Clinical discussion: EDCs can occur anywhere in the GI tract, most frequently in small intestine and least often in cecum. MD is true diverticulum of small intestine. This case report discusses a rare occurrence of a pediatric patient having both an EDC and MD, emphasizing diagnostic and management challenges in such unique cases. Conclusion: Clinicians should be aware about simultaneous occurrence of EDC and MD in pediatric population who may present as intestinal obstruction.