Midgut malrotation and incomplete rotation are common causes of neonatal intestinal obstruction. At end of 10 week of intrauterine life, cecum will be placed in subhepatic region temporarily and descends to right lower quadrant by eleventh week. Arrest of cecum in subhepatic region or undescended cecum is a rare congenital anomaly of mid gut. Usually, it remains asymptomatic and is diagnosed incidentally. If any pathology occurs in anomalous part, like appendicitis then the diagnosis and treatment will be challenging in all age groups. Variation in blood supply have also been reported with anomalies leading to iatrogenic injuries during colonoscopy and surgeries. Lack of knowledge of these rare variations may lead to delayed diagnosis of appendicitis leading to perforation and surgical emergencies. In the present case, we describe an undescended cecum and its associated variation in branching pattern of superior mesenteric artery.