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对2019年6月贵阳市儿童医院收治的1例先天性支气管-胆管瘘(CBBF)患儿的临床资料进行回顾性分析。患儿，女，7岁6个月，临床表现为咳嗽伴咳大量黄绿色黏痰，出生后反复肺部感染为主要临床表现，通过电子支气管镜发现瘘口后，与影像科、麻醉科、儿外科多学科协作，经治疗患儿康复出院。CBBF报道较少，针对儿童出生后即出现反复肺部感染伴黄绿色痰液，抗感染治疗效果不佳的难治性肺炎应警惕CBBF，尽早行支气管镜检查并联合影像学技术早期诊断，多学科协作下制定合理诊疗方案，提高对此类罕见病的临床诊治水平，减少漏诊及误诊。“,”To retrospectively analyze the clinical data of a child with congenital broncho-bile duct fistula(CBBF) in Guiyang Children′s Hospital in June 2019.A female, aged 7 years and 6 months old, patient presented cough with a large amount of yellow green mucus.The main clinical manifestation was recurrent pulmonary infection after birth.After the fistula was found by electronic bronchoscope, doctors cooperated with imaging department, anesthe-siology department and pediatric surgery department.After treatment, the child recovered and discharged.There are few reports on CBBF.This study suggested that, in view of the refractory pneumonia with recurrent pulmonary infection and yellow green sputum after birth, and that the effect of anti-infection treatment was poor, clinicians should pay attention to the CBBF, take bronchoscopy as soon as possible, and make early diagnosis by combining with imaging technology, thus formulating a reasonable diagnosis and treatment plan under multidisciplinary cooperation, so as to improve the diagnosis and treatment of this rare disease clinical diagnosis and treatment level, and reduce missed diagnosis and misdiagnosis as well.