Introduction: Concurrent presence of two distinct pathologies, such as acute mucosal appendicitis with epiploic appendagitis, is unusual and can often lead to a management dilemma for surgeons. Here, we present a case description of this clinical rarity and reviewed the available literature in managing this rather uncommon but potentially life-threatening clinical condition. Case Report: A 44-year-old male presented to the emergency department with a one-day history of nonmigratory acute right iliac fossa pain. Clinical examination revealed right iliac fossa tenderness with positive rebound sign. Biochemical tests were generally insignificant. Abdominal computed tomography (CT) showed features suggestive of acute appendicitis (diameter of appendix was 7 mm and inflammatory changes noted in the mid part). Based on these findings, a laparoscopy was performed. Intraoperatively, there was a macroscopically normal appearing appendix and an anticlockwise rotation of necrotic epiploic appendage. The necrotic tissue was transected followed by a planned appendicectomy. The histology confirmed the presence of both epiploic appendagitis and