BACKGROUND : Patients with appendicular endometriosis (AE) constitute <1% cases of all pelvic endometriosis cases. AE presents with pain in right iliac fossa and symptomatically mimics appendicitis and definitive diagnosis is possible only after histopathological examination of excised appendix. METHODS : In this case report, we present a rare case of appendicular endometriosis in a young woman suffering from pain in right iliac fossa and periumbilical region (on/off) for the past one year. She had a past history of infertility. Blood investigations were normal and CT scan of abdomen demonstrated findings suggestive of appendicitis. Laparoscopic appendicectomy was performed, which revealed 2 cm tumor-like mass (1.5 cm diameter) present at the tip of appendix involving the mesoappendix. There was no ascites, peritoneal or omental deposits or any signs of inﬂammation. Post-operative recovery was good. Dissected appendix specimen was sent for histopathological examination which confirmed endometriosis of appendix. CONCLUSIONS : Gastrointestinal endometriosis (GE) accounts for 3-37% of all endometriosis cases, whereas AE is present in only ∼3% of all GE cases and constitutes <1% of all the endometriosis cases. AE frequently involves tip and body of the appendix. The layers of appendix most commonly affected are muscular and seromuscular (∼2/3rd cases), followed by the serosa (∼1/3rd cases). CONCLUSIONS : Thus, appendicular endometriosis is a rare entity and should be included in differential diagnosis in young women presenting with pain in right iliac fossa and having history of infertility.