Acute appendicitis is one of the most common reasons for presentation to the emergency department that requires an emergency appendectomy. Clinical presentation with abdominal pain in the left lower quadrant is very uncommon but can occur with a congenital left-sided appendix or right-sided long appendix. We report a rare case of a 65-year-old man with incidental finding of situs inversus totalis who presented with left lower quadrant abdominal pain. A CT scan of the abdomen confirmed the diagnosis of left-sided acute appendicitis, and the patient underwent laparoscopic appendectomy with an uneventful postoperative course.