Importance:Congenital tuberculosis(TB)is a rare,potentially fatal disease.There is currently a lack of detailed clinical information available regarding this disease.Objective:This retrospective study investigated the clinical manifestations,treatment,and long-term prognosis of congenital TB.Methods:Patients were treated in Beijing Children's Hospital,Capital Medical University(Beijing,China)between 2009 and 2018.Their demographic data,maternal and family histories,symptoms and signs,treatment information,and follow-up data were retrospectively collected using the hospital's electronic information system.Results:Ten infants with congenital TB were enrolled.The mean gestational age was 36.6±2.2 weeks and mean birth weight was 2517±487 g.All 10 patients exhibited fever,nine patients(90％)had anemia,and six patients(60％)had extrauterine growth retardation.On chest computed tomography scans,all 10 patients presented multiple pulmonary nodules and four patients(40％)had mediastinal adenopathy.Nine out of ten(90％)completed the T-spot test,and eight of them(8/9,89％)were positive.Anti-TB treatment was initiated upon diagnostic confirmation.All patients(100％)received combined treatment with isoniazid(INH)and rifampicin(RIF).Eight of 10 patients(80％)received combined treatment with INH,RIF,and pyrazinamide.The survival rate was 100％.One patient was lost to follow-up and four patients are currently continuing treatment.Three of nine patients(33％)achieved normal developmental milestones at 6 months of age.Interpretation:Early diagnosis based on maternal history,typical imaging results,and timely treatment can improve outcomes in infants with congenital TB.