Endometriosis of the appendix is a very rare entity and commonly affects females in childbearing age. Clinical presentation might be confusing varying from asymptomatic to acute abdominal pain and often mimicks acute appendicitis or chronic pelvic pain. Diagnosis is generally made after pathological examination as operative findings are usually non-specific. This condition poses a diagnostic challenge to radiologists and surgeons altogether and we therefore report a case of a middle aged female who presented with both right lower quadrant and right lower back pain. Recent literature is reviewed and radiological findings discussed.