<div><p>Abstract Endometriosis can have several different presentations, including overt ascites and peritonitis; increased awareness can improve diagnostic accuracy and patient outcomes. We aimto provide a systematic review and report a case of endometriosis with this unusual clinical presentation. The PubMed/MEDLINE database was systematically reviewed until October 2016. Women with histologically-proven endometriosis presenting with clinically significant ascites and/or frozen abdomen and/or encapsulating peritonitis were included; thosewith potentially confounding conditionswere excluded.Our search yielded 37 articles describing 42 women, all of reproductive age. Ascites was mostly hemorrhagic, recurrent and not predicted by cancer antigen 125 (CA-125) levels. In turn, dysmenorrhea, dyspareunia and infertility were not consistently reported. The treatment choices and outcomes were different across the studies, and are described in detail. Endometriosis should be a differential diagnosis of massive hemorrhagic ascites in women of reproductive age.</p></div
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