Background: Endometriosis-related pleural effusion (PE) is a relatively rare but treatable cause of bloody PE. The clinical characteristics and outcome of patients with endometriosis-related PE remain unknown. Objectives: We present a case of endometriosis-related PE diagnosed on pleural fluid cytology. A systematic review of all endometriosis-related PE cases in literature was conducted to elucidate the clinical characteristics, explore the diagnostic yield of pathological examinations, and to summarize the outcomes of therapeutic approaches in this disease. Methods: A case of endometriosis-related PE diagnosed in study hospital was reported. PubMed, Web of Science, and EMBASE were searched for publications pertaining to cases of endometriosis-related PE using predefined search terms. This case and those identified from PubMed, Web of Science, and EMBASE were analyzed. Results: A total of 67 patients were included. Catamential symptoms were presented in 30 (44.8%) patients. Dyspnea was presented in 50 patients (74.6%), followed by right chest/shoulder pain in 34 (50.7%) and cough in 18 (26.9%). 82.8% of the patients had concomitant pelvic endometriosis and 76.7% was infertile or nulliparous. The diagnostic yield of pleural fluid cytological examination, percutaneous pleural biopsy, and surgical biopsy was 9.0, 45.5, and 78.7%, respectively. The patients who received surgery-based therapy had a significantly longer time to relapse than those who received progestational agents or GnRH analogs alone (P = 0.025) or hysterectomy and bilateral salpingoophorectomy (HBSO) (P = 0.040). Conclusions: High clinical awareness of pleural endometriosis is essential in all female with hemorrhagic PE, especially in young females who have infertility and/or pelvic endometriosis. Plerual fluid cytology might be a simple minimally invasive and cost-effective modality in the diagnosis of endometriosis-related PE. Treatment is challenging due to high recurrence and the optimal management of endometriosis-related PE needs further evaluation. The combined approach by surgery and hormonal therapy may achieve the best relapse-free survival.