Tuberculosis is one of the leading infectious diseases in the world and is still a serious public health problem in many countries. Congenital tuberculosis is a rare but fatal condition if left untreated. In this report, three-month old boy who admitted to our clinic with respiratory distress, fever and failure to thrive still after birth, diagnosed as congenital tuberculosis. He had massive hepatosplenomegaly, pulmonary involvement with cavitation and central nervous system involvement. Her mother had active tuberculosis during pregnancy. With anti-tuberculous therapy, clinical remission was excellent. In the infancy period, therapeutic approachments against congenital or postnatally acquired tuberculosis were similar. If tuberculosis was diagnosed in infancy period, systemic evaluation and family surveillance, especially mother, for tuberculosis must be performed.