Enterobius vermicularis infestation is a global public health concern. The exact pathogenesis of Enterobius species causing acute appendicitis is debatable and only a few cases have been reported in India and different countries of the world. Recognition of worms inside the appendix is important as it affects the definitive management of the patients depending on the stage of the disease. Here, intend to present such a rare case of acute appendicitis in association with Enterobius vermicularis parasite. A 46-year-old male patient presented to the Emergency Department with complaints of right lower abdominal pain and nausea. Investigations were done and the patient was clinically suspected of acute appendicitis. Appendicectomy was performed and grossing of the specimen was done according to the protocol. Histopathological examination revealed ulcerated mucosa with evidence of transmural inflammation. Lumen of the appendix showed Enterobius vermicularis worm containing multiple eggs. High worm burden can cause appendiceal obstruction and other devastating complications. Detailed clinical-pathological evaluation is necessary to identify the etiology in a case of acute appendicitis. This helps in the definitive management of the patient and uncomplicated cases may be managed with conservative treatment. © 2025 Elsevier B.V., All rights reserved.