INTRODUCTION: Autoimmune hepatitis (AIH) is a relatively infrequent and complex liver disease characterized by acute or chronic inflammation, interface hepatitis in histology examination, elevation of immunoglobulin G (IgG), production of autoantibodies, and is often responsive to immunosuppression. The incidence of AIH has been increasing worldwide, affecting people of all ages and sexes. AIH represents a diagnostic challenge because of its heterogeneous presentation and the lack of pathognomonic findings. Even when treated, AIH can remain a progressive disease. In this review, we present recent data on the natural history of AIH and the developing evidence on the management of patients with AIH. AREAS COVERED: This review outlines the clinical presentation, risk factors linked to poorer clinical outcomes, the diagnostic algorithm, and the current management strategies for individuals living with AIH. EXPERT OPINION: AIH remains a clinical challenge, and new tools for better diagnosis and stratification of risk are needed. In addition, better treatments are needed as a complete response is achieved in less than 60% of cases, and intolerance to first-line treatment is frequent. The use of biological treatment in AIH seems to improve the response rate and minimize the risk of side effects of current medication in this increasingly prevalent disease.