Appendicitis is the most common condition causing acute abdomen. The relationship of parasitic infestation with acute appendicitis has been a controversial issue. A 41-years-old female patient presented with abdominal pain in the right lower quadrant for 48 hours, followed by nausea and vomiting. She underwent laparotomy with a McBurney's incision. We could identify appendicitis with the presence of an Ascaris lumbricoides worm occupying the appendiceal lumen and extending to the cecum and ileocecal valve. In our opinion Ascaris lumbricoides with acute appendicitis is rare clinical case; especially for nonendemic areas. Even if it is a rare condition, it should be kept in mind.