Acute appendicitis is the most common intra-abdominal pathology that requires emergency surgery in general surgery clinics. The aetiology of acute appendicitis includes both infectious and non-infectious causes. Although parasitic diseases are rare in developed countries, they are more common in developing countries. The association between acute appendicitis and parasitic infections is unclear, and whether parasites are the cause of acute appendicitis is still under debate. In addition, many appendix parasites are encountered after appendectomy. In this study, we report a rare case of acute appendicitis caused by Enterobius vermicularis.