OBJECTIVE: Considering the rarity and the significance of early diagnosis for planning appropriate treatment, a case of congenital tuberculosis is reported.CASE REPORT: After a prolonged stay in the Neonatal Intensive Care Unit (NICU) of a male pre-term infant without improvement, the diagnosis of tuberculosis was suspected when his mother underwent thoracotomy for valve replacement due to a subacute endocarditis. At this time, multiple pleural granulomas were detected and the diagnosis of tuberculosis was confirmed by histology. His mother seldom went to the NICU to see the baby and she was rarely very close to him in these occasions. After tuberculosis therapy, the baby gradually improved. CONCLUSION: Among the possible ways of transmission, the reported case was probably transmitted by aspiration of contaminated amniotic fluid in utero or at the time of delivery.