Congenital tuberculosis is a rare disease and it is difficult to diagnose because of the nonspecific nature of the presenting symptoms, signs and the fatal outcome in the absence of early therapy. We experienced a case of congenital tuberculosis in a newborn infant, who was born prematurely to a mother who had a miliary pulmonary tuberculosis confirmed on the 34th postpartum days. At the age of 32 days, chest X-ray revealed total hazziness of the right lung and the infant required intubation and mechanical ventilation. At that time a significant number of fungi were found in the urine culture. Amphotericin B therapy was started with the impression of fungal pneumonia. DIC developed despite of intensive therapy. Based on the medical history of the mother, laboratory tests were done on the infant for the isolation of M. tuberculosis at the age of 34 days. Acid-fast bacilli were seen in the gastric aspirates and sputum by staining and antituberculosis therapy with isoniazid, rifampin, pyrazinamide and prednisolone was started. Significant clinical improvement occurred 2 weeks after therapy began.