Perinatal tuberculosis can be divided into congenital tuberculosis due to intrauterine infection and neonatal tuberculosis due to infection irnmediately following birth. It is a rare disease entity with only 300 cases reported worldwide. In Korea, only a few cases have been reported and only 1 case has been confirmed by autopsy at neonatal period. Although the majority of the mothers are found to have advanced tuberculosis and the children themselves are usually of premature birth, early diagnosis is difficult and despite antituberculous medication, the mortality rate is high. We report a premature baby with respiratory difficulty admitted to our hospital, whose mother was found to have miliary tuberculosis during the course of management. The baby died and autopsy was performed to confirm congenital tuberculosis.