Hepatocellular adenoma (HCA) is a rare benign liver tumor in the pediatric population, often associated with underlying genetic syndromes or metabolic disorders. In prepubertal children, the most common subtypes are β-catenin–mutated HCA and HNF1α-inactivated HCA (H-HCA), each with distinct clinical and prognostic implications. Differentiating HCA from malignant lesions such as hepatocellular carcinoma (HCC) is critical for appropriate management. We report a 2-year-old girl with hepatic failure, who was found to have multiple hepatic nodules. Imaging studies suggested features highly indicative of HCC. Due to multiple suspicious lesions and severe liver dysfunction, liver transplantation (LT) was performed. Histopathological examination confirmed the lesions as H-HCAs. The patient was postoperatively diagnosed with hereditary tyrosinemia type 1. This case highlights the importance of including HCA in the differential diagnosis of hepatic nodules in children with metabolic disorders. Accurate diagnosis of HCA is essential to guide clinical decision-making, optimize treatment strategies including LT.
