Autoimmune hepatitis is a consequence of a triggering antigen and genetic factors that favor the presentation of autoantigens, polymorphisms that affect immunocyte activation and durability, cytokine alterations that promote proliferation of liver-infiltrating cytotoxic T cells, and perturbations in the number and function of immune-regulatory cell populations, including T regulatory cells and natural killer T cells. The triggering epitope is probably a short sequence peptide that is common in multiple infectious or toxic agents. Homologies between this epitope and self-antigens (molecular mimicry) may stimulate humoral and cellular responses that are cross-reactive. Sensitized immunocytes extend and perpetuate the inflammation through imprecise targeting of self-antigens that resemble foreign antigens (promiscuous behavior). The occurrence and clinical phenotype of the disease may relate to genetic susceptibility factors that favor protracted exposure to indigenous etiological agents, and these genetic factors can vary in different geographical regions and ethnic groups. The clinical phenotype within a population can be modified further by genetic polymorphisms that are not disease specific and that affect immunocyte activation, differentiation, proliferation and programmed death (apoptosis). Autoimmune hepatitis is a model of autoreactivity that reflects multiple disturbances in the counter-regulatory mechanisms essential for immune homeostasis.