Congenital tuberculosis is a rare disease, difficult to be correctly diagnosed from clinical manifestations alone without maternal tuberculous history. In this article we report a neonate who exhibited abdominal distension and bloody diarrhea simulating neonatal sepsis and necrotizing enterocolitis. Exploratory laparatomy was performed for the markedly distended abdomen and deteriorated condition. Calcified lymph nodes over mesentery and spleen were found, and biopsy was done to show caseous tubercles. However, this neonate deteriorated rapidly and died one day after operation. Autopsy revealed generalized discrete miliary tubercles over the lungs, liver, spleen, pancreas, adrenal glands, thyroid and lymph nodes.