INTRODUCTION: Appendico-ileal knotting is an extremely rare complication of acute appendicitis resulting in small bowel obstruction (SBO) and potential strangulation. It accounts for a negligible fraction of SBO cases, with only a handful of cases reported globally in the literature. The condition arises when an inflamed or gangrenous appendix forms a knot around the ileum, often exacerbated by adhesions or anatomical variations, leading to closed-loop obstruction and, if untreated, bowel strangulation and gangrene. CASE PRESENTATION: A 34-year-old female presented with four days of crampy mid-abdominal pain, nausea, bilious vomiting, and absence of bowel movements. Examination revealed generalized peritonitis, diffuse tenderness with guarding, and rigidity, accompanied by leukocytosis. An emergency laparotomy uncovered hemorrhagic fluid and an inflamed appendix wrapping around the distal ileum, causing a closed-loop obstruction with necrotic-appearing bowel. Following hemicolectomy, ileostomy, and appendectomy, the patient stabilized, was weaned off vasopressors, and recovered uneventfully. Discharged on postoperative day seven and followed up regularly, she eventually underwent ileostomy reversal three months later without complications. DISCUSSION: Acute appendicitis rarely leads to SBO through appendico-ileal knotting. Preoperative diagnosis is challenging due to its rarity and non-specific radiologic features. Surgical management varies from simple appendectomy to resection of gangrenous bowel, with decisions guided by intraoperative findings. CONCLUSION: High clinical suspicion, prompt recognition, and timely surgical intervention ensure better outcomes in appendico-ileal knotting.