A 3-month old male patient, with chronic constipation and abdominal distension complaints, was intraoperatively diagnosed with ileocecal stenosis with agenesis of the vermiform appendix. Agenesis of the vermiform appendix is a very rare anatomical finding with an incidence rate about 0.001% in patients who receive laparotomy due to suspected acute appendicitis. No previous cases of ileocecal stenosis with agenesis of the vermiform appendix were reported in the literature. Ileocecal stenosis with agenesis of the vermiform appendix, which present with more shallow symptoms as opposed to ileocecal atresia, can be a cause of congenital bowel obstruction during neonatal period. Stenotic segment was resected without ileocecal valve reconstruction and then ileocolic anastomosis was performed. Development of the baby was normal during post-op 6-month follow-up period. The main objective of this case report is to attract the attention of pediatric surgeons in differential diagnosis of ileus and treatment strategies during laparotomy. (C) 2018 Anatomical Society of India. Published by Elsevier, a division of RELX India, Pvt. Ltd. All rights reserved.