BACKGROUND: Bowel obstruction is a common surgical emergency worldwide. It may result into high morbidity or mortality whenever intervention is delayed. It affects all age groups. The most commonly seen etiologies of bowel obstruction are post-operative adhesions, neoplasm and hernia in that order. Intestinal knot syndrome is an extremely rare cause of intestinal obstruction, and when it occurs, it poses diagnostic challenges. We report a case of appendico-ileal knotting causing strangulated small bowel obstruction due to its rarity and diagnostic difficulty. Our objective is to discuss the clinical presentation and management of this rare cause of surgical emergency. CASE: A-72- year old man was seen at the emergency unit of our center with 4 days history of gradual onset of colicky abdominal pain with nausea and vomiting. He had a two-day history of constipation and a one-day history of fever. He was acutely ill-looking and his vital signs were abnormal. Urgent abdominopelvic ultrasound and plain abdominal x-ray was performed and were suggestive of intestinal obstruction. He had emergency laparotomy, and intra-operatively appendico-ileal knotting was seen with gangrenous appendix and terminal ileum. This necessitated limited right hemicolectomy and ileo-colonic anastomosis. The patient was managed post-operatively and discharged on post-operative day 10. CONCLUSION: Appendico-ileal knotting is a cause of small bowel obstruction although it is very rare. The diagnosis is commonly confirmed intra-operatively. There are reports of simple small bowel obstruction secondary to appendico-ileal knotting, but this case confirmed that it could also lead to strangulated intestinal obstruction.