RESUMEN
Autoimmune hepatitis [AIH] is rare in children. Two types of childhood autoimmune hepatitis are recognized: AIM type 1 which is characterized by the presence of smooth muscle [SMA] and/or antinuclear [ANA] antibodies; and AIH type 2 which is positive for anti-liver kidney microsomal type 1 [anti-LKM-1] antibody. Anti-mitochondrial antibody [AMA] is considered the hallmark for primary biliary cirrhosis [PBC] that occurs primarily in adult women and is characterized by destruction of the intralobular bile ducts and progression to cirrhosis and liver failure. Antimitochondrial-antibody-positive AIH is extremely rare in children. We report a 13 year old Saudi girl with type-1 AIH who had a strongly positive anti-mito-chondrial antibody and no evidence of small bile duct disease in the liver biopsy