Endometriosis associated with ascites is a rare form of endometriosis. We identified only 81 publications describing women with this pathology. The purpose of this review was to search the existing literature and to understand the clinical presentations and possible treatment options. A 28-year-old patient was admitted due to a fever of unknown origin and lower abdominal pain. Her CT scan was normal with some non-specific lymph-node enlargement up to 1.6 cm and the presence of ascites. Diagnostic laparoscopy revealed a large amount of hemorrhagic ascites. The entire peritoneal surface was covered with milliary nodules. The pathology report revealed peritoneal stromal endometriosis. Endometriosis is an important differential diagnosis in women with ascites and treatment of this form of endometriosis is a real dilemma.
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