Abstract
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an inborn error of immunity, resulting from variation in the autoimmune regulator gene (AIRE). Pathogenic variants in the AIRE gene result in autoimmunity typically involving endocrine organs with nonendocrine organs less commonly affected. Hepatitis associated with APECED has emerged as a potentially fatal complication with higher reported prevalence in the Americas. We describe a case of a 3-year-old boy presenting with hepatitis from APECED without classical clinical diagnostic criteria. This case highlights the importance of APECED in the evaluation of hepatitis given response to immunomodulator treatment and risk of fulminate liver failure.