In 1969, Sharp described the first cases of aplhai-antitrypsin deficiency disease in children with juvenile liver cirrhosis. Since then, this inborn error has been recognized as one of the more common factors in cirrhosis of infancy and childhood, especially in Caucasians of Northern Europeans. PiZZ phenotype is the only one associated with liver disease, there have been a few rescent reports of cirrhosis in heterozygous patients. We experienced a case of 24-year-old male patient who was diagnosed as liver cirrhosis by biopsy but had no defintie causes of liver cirrhosis. The laboratory findings were compatible with liver cirrhosis. By biopsy, the liver is composed of variable sized, micro and macronodules. Dense fibrous tissue encase these nodules. The hepatocytes are strongly positive granules and diastase resistant. Pi phenotype was Pi MIZ by IEF (immunoelectric Focusing) method and the plasma alphai- antitrypsin level is lower than normal.