Enterobius vermicularis is one of the most common parasites in the world and the infection rate is higher in children than adults. Although the most common clinical manifestation is perianal pruritus, it may cause other gastrointestinal manifestations. We report one case of appendicitis caused by Enterobius vermicularis. The clinical difference between typical appendicitis and the appendicitis due to Enterobius vermicularis is unclear. The patient had a repetitive Enterobius vermicularis infection for a past year, which was treated with occasional vermicides only. It is considered that the appendicitis is developed from the intractable Enterobius vermicularis infection.