Endosalpingiosis is occasionally incidentally found on histopathologic examination of gynecologic or gastrointestinal specimen, most commonly in the ovary, fallopian tube, omentum and uterus. Recently an association between endosalpingiosis and, ovarian and uterine cancer has been described. Here, we describe a rare case of appendiceal endosalpingiosis mimicking appendicitis. Further prospective studies are required to elucidate the clinical significance of appendiceal endosalpingiosis, the potential association with gynecologic malignancy and implications for management.