The case of a young female patient with homozygous alpha-1-antitrypsin deficiency (Pi-ZZ) associated chronic liver disease, who developed primary hepatic tuberculosis shortly after delivery of a healthy baby girl is reported. These findings emphasize that this rare disease should be considered even in patients with precirrhotic liver disease, while pointing out that the genetically determined deficiency of protease inhibitor alpha-1-antitrypsin (AAT) predisposes to hepatic infection.