BACKGROUND Ileo-cecal lipomatosis is a rare, benign condition characterized by diffuse submucosal adipose tissue infiltration in the intestinal wall. While usually asymptomatic, it occasionally presents with complications such as bowel obstruction, perforation, or mimicry of acute appendicitis, often leading to diagnostic challenges. CASE REPORT A 29-year-old obese woman presented to the Emergency Department with acute abdominal pain, nausea, vomiting, and fever, clinically suggestive of acute appendicitis. Examination revealed right iliac fossa tenderness, and laboratory findings showed elevated white blood cell counts. Computed tomography (CT) imaging indicated an inflammatory mass in the ileo-cecal region, raising suspicion for appendicitis with possible abscess formation. During emergency laparotomy, diffuse thickening and lipomatous infiltration of the ileo-cecal junction were identified rather than a ruptured appendix. Ileo-cecal resection was performed, and histopathological analysis confirmed diffuse submucosal lipomatosis without malignancy or evidence of true inflammatory appendicitis. CONCLUSIONS This case emphasizes the importance of considering rare conditions like intestinal lipomatosis in the differential diagnosis of acute abdominal pain, particularly in atypical presentations. While radiological findings may suggest common conditions like appendicitis, they may be inconclusive, necessitating surgical exploration. Early recognition of ileo-cecal lipomatosis is essential for timely and appropriate management, reducing the risk of unnecessary procedures or complications. Increasing awareness of this rare entity among clinicians can improve diagnostic accuracy and patient outcomes.