Endometriosis in a patient with Rokitansky-Kuster-Hauser syndrome

Endometriosis in Rokitansky-Kuster-Hauser syndrome has been reported only once previously. We present here a case of endometrioma in a patient with Rokitansky-Kuster-Hauser syndrome. A 26-year-old patient with Rokitansky-Kuster-Hauser syndrome presented with abrupt pain in the left iliac quadrant. A mass was confirmed by sonography. Laparoscopic inspection revealed no uterus. The mass was removed laparoscopically. Endometriosis was confirmed histologically. If endometrioma in a patient with Rokitansky-Kuster-Hauser syndrome is associated with a small rudimentary unicornis uterus with a small endometrial cavity, endometriosis can be assumed to have developed by retrograde menstruation theory. Because the patient did not have a functioning endometrium, her endometrioma is assumed to have arisen from coelomic metaplasia.