Perinatally acquired neonatal tuberculosis occurs rarely, is difficult to diagnose, may be the indicator of untreated tuberculosis in the mother, and could result in nosocomial transmission to neonatal patients, visitors to neonatal intensive care units, and health care workers. The disease may be more common in certain ethnic and social groups. Neonatal mortality approaches 30%. We report two cases with different outcomes. A neonate was treated for clinical miliary tuberculosis and survived; Mycobacterium tuberculosis was cultured from bronchoscopic washings, maternal genital fluids, and tissues. A second infant died at age 46 days, and autopsy disclosed miliary tuberculosis of lungs, mediastinal and mesenteric nodes, liver, spleen, and bone marrow. The lungs were most severely affected, but the placenta and central nervous system were not involved. The histopathology was not granulomatous. After the diagnosis in the infant, the mother was ascertained to have pulmonary and genital tuberculosis. Fetal and neonatal tuberculosis could be acquired transplacentally as prenatal tuberculous chorioamnionitis, perinatally through aspiration and ingestion of infected maternal genital tissues and fluid, or postnatally through droplet spread from cases of active tuberculosis. These two neonates probably acquired the disease perinatally from maternal genital tuberculosis.