Leiomyoma is a benign tumor of smooth muscles and can essentially arise from any part of the body that contains smooth muscles. It is more commonly found in the uterus, while those from the gastrointestinal tract are rare. The spectrum of clinical presentation is rather wide, from a vague abdominal discomfort or acute abdomen mimicking more common diagnoses such as acute appendicitis or acute gastroenteritis to features of intestinal obstruction and gastrointestinal bleeding. Here, we share our experience with a case of a young woman whose symptoms and signs were typical of acute appendicitis, which intraoperatively turned out to be a twisted mesenteric tumor located at 70 cm from duodenojejunal flexure. The tumor was managed by segmental resection and anastomosis. She had an uneventful recovery postoperatively, and histopathological examination of the surgical specimen was consistent with an infarcted mesenteric leiomyoma. The rarity of this entity mimicking appendicitis is highlighted, and should it never get twisted, it would have probably remained unnoticed for the rest of her life. In the current era of laparoscopy, it is always wise to never skip a thorough examination of the whole length of the small bowel in order to locate this type of pathology.