The origin of endometriosis has multiple theories, with controversy over which may demonstrate the prominent pathophysiology. The most common extra-pelvic organ system affected by endometriosis is the gastrointestinal tract. Gastrointestinal endometriosis (GE) accounts for 3 to 37% of all endometriosis cases, and appendiceal endometriosis is present in around 3% of GE cases, therefore constituting less than 1% of all endometriosis cases.In this report, we present a 24-year-old female with a past medical history significant for endometriosis status post two excisional laparoscopies who presented with eight months’ duration of right lower quadrant pain, constant and stabbing, with rebound tenderness. Appendectomy and histopathology demonstrated focal endometriosis, diffuse serosal fibrovascular adhesions involving the appendiceal serosa/subserosa, as well as a dilated lumen filled with hemorrhagic content.When the appendix is not considered in endometriosis pathology, patients are at increased risk for unresolved pain and further laparoscopic procedures. Prophylactic appendectomy appears to be a worthwhile consideration in patients with chronic pelvic pain, given the high frequency of appendiceal pathology.