先天性结核病是胎儿在子宫内或分娩时感染结核分枝杆菌而引起的结核病变，是一类较特殊、罕见的儿童结核病。先天性结核病的临床表现缺乏特异性，早期诊断困难、进展迅速、易误诊、死亡率高。目前国内对先天性结核病的报道和研究较少，许多医生对该病临床认识及诊疗经验不足。该文通过对先天性结核病流行病学、临床表现进行简要总结，并结合现有研究对先天性结核病的快速病原学检测及诊断与治疗进展进行综述，以加强临床医生对该病的认识。“,”Congenital tuberculosis is a tuberculosis disease caused by the fetus infected with Mycobacterium tuberculosis in the womb or during delivery, which is a kind of special and rare tuberculosis in children.The clinical manifestation of congenital tuberculosis is lack of specificity, difficulty in early diagnosis, rapid progress, easy misdiagnosis and high mortality.At present, there are few studies on congenital tuberculosis.In this review, we will discuss the epidemiology and clinical features of congenital tuberculosis.Importantly, we will further introduce the rapid etiological detection and treatment to strengthen clinicians′ understanding of congenital tuberculosis.