The presence of ascites is a common clinical presentation in gynecologic oncology patients. Hemorrhagic ascites (HA) due to endometriosis is a rare presentation that can be easily misdiagnosed as ovarian malignancies. The present study aims to update the currently available knowledge on the characteristics of patients presenting with HA due to endometriosis.A systematic search was conducted for articles published from January 2000 to July 2020 using the Medline, Scopus, and Google Scholar databases along with the references of the full-text articles retrieved. Papers describing cases of women over 18 years with or without previous history of endometriosis were assessed. Only cases with histologically proven hemorrhagic ascites of endometriosis origin were included.Twenty-nine studies (27 case reports and two case series) comprising 32 patients were evaluated. The mean patients’ age was 32 years, while six of the patients had a previous history of endometriosis. The mean amount of drained ascitic fluid was 4,200 mL, whereas three patients underwent thoracentesis due to pleural effusions. The treatment options included not only medical but also surgical therapies. Fertility preservation was achieved in 27 patients, while two of them achieved pregnancy with in vitro fertilization (IVF) techniques.Endometriosis-related hemorrhagic ascites is a relatively rare expression of the disease. Endometriosis-related hemorrhagic ascites should be considered in the differential diagnosis (DD) of women with ascites and clinical suspicion of endometriosis. The available literature is limited to case reports and case series and thus indicates further research in the field to decode the pathophysiology of the disease and decide on the optimal treatment.