α₁-antitrypsin deficiency is a genetic disorder associated with liver disease mainly during infancy or childhood and with emphysema in adults. It is the most common metabolic disease as an indication for liver transplantation in children. Liver injury is observed only in 10-15% of children with homozygous form of α₁-antitrypsin deficiency. The natural course of the disease is variable and pathogenesis of liver changes is not clear. Liver transplantation was performed at the age of 1-17 years in our patients. The rapid progress of liver injury in two infants, seldom observed at this age, is reported in this paper.