Epidermoid cysts are benign cystic lesions that are usually found in the skin. They can also be found in the organs of the gastrointestinal system. Here, we report a rare case of epidermoid cyst of the appendix which has been published in only three cases in the literature. They can be either congenital or acquired. Congenital epidermoid cysts are related to the inclusion of ectodermal elements at the time of neural groove closure. Acquired cysts are thought to be due to trauma or iatrogenic implantation of the epidermis in locations favorable to growth during surgery. Diagnosis is mainly by histopathological examination of the tissue sample. Complete excision of the cyst with the walls intact is considered curative.