目的:报告3例不同原因引起的非创伤性乳糜胸病例并进行文献复习，以期提高对非创伤性乳糜胸临床特点和诊治方法的认识。方法:对南京大学医学院附属鼓楼医院收治的3例非创伤性乳糜胸，包括胸部淋巴管畸形相关2例、非结核性感染相关1例进行回顾，同时以“淋巴管瘤”加“乳糜胸”“淋巴管畸形”加“乳糜胸”“感染”加“乳糜胸”为关键词在CNKI和万方数据库上搜索，并以“lymphatic malformation”AND“chylothorax”“lymphangiomatosis”AND“chylothorax”“infection”AND“chylothorax”在PubMed进行搜索并筛选。结果:在淋巴管畸形相关的案例中小于18岁6例，大于等于18岁5例，男6例，女5例，发热3例，不发热8例，首发症状中均有胸闷胸痛等胸腔积液表现。乳糜液性状中有记载的4例为血性，1例为乳白色。治疗方法中7例手术治疗，1例放疗，2例药物治疗，1例自动出院后失访。转归中6例存活，3例死亡，2例不详。在感染相关的乳糜胸案例中，男2例，女2例；3例乳糜胸乳糜液呈乳白色，均以药物治疗；4例均无乳糜胸致死的报告。结论:非创伤性乳糜胸相对不常见，非乳白色的胸水依然需要警惕乳糜胸的可能。非创伤性乳糜胸病因复杂多样，预后与病因可能相关，胸部淋巴管畸形所致乳糜胸相比非结核感染性乳糜胸预后差，且更多的需要手术干预。“,”Objective:Report three cases of non-traumatic chylothorax caused by different reasons and review the literature, in order to improve the understanding of the clinical characteristics and diagnosis and treatment of non-traumatic chylothorax.Methods:Three cases of non-traumatic chylothorax from Drum Tower Hospital Affiliated to Medical School of Nanjing University, including two cases related to thorac lymphatic malformation and one case related to non-tuberculous infection, were reviewed.At the same time, “lymphatic malformation” AND “chylothorax” “lymphangiomatosis” AND “chylothorax” “infection” AND “chylothorax” were retrieved and screened in the CNKI Medical database and Wanfang Medical database in Chinese and in PubMed database in English respectively.Results:Among the cases related to lymphangiomatosis, six cases were less than 18 years old, five cases were 18 years old or older, six cases were male, five cases were female, three cases were febrile, eight cases were non febrile.The first symptoms included chest distress and chest pain.There were four cases of bloody and one case of milky white in hydrothorax.Among the treatment methods, seven cases were treated by operation, one case by radiotherapy and two cases by medication, the remain case was discharged with out targeted treatment.Among these cases, six cases survived, three cases died and two cases were unknown.In the cases of chylothorax associated with infection, two cases were male, two cases were female.There were three cases of milky white hydrothorax.All of them were treated with drugs.There were no report of chylothorax death was found in four cases.Conclusions:Nontraumatic chylothorax is relatively uncommon, non milky white pleural fluid still needs to be vigilant about the possibility of chylothorax.The causes of nontraumatic chylothorax are complex and diverse.The prognosis may be related to the causes.The prognosis of chylothorax caused by thorac lymphotic malformation is worse than that of non-tuberculosis infectious chylothorax, and more surgical intervention is needed.