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Highlights • Hepatocellular carcinoma (HCC) is an extremely rare finding in young adult. • Giant HCCs are larger than 10 cm in diameter and mostly develop in non-cirrhotic livers. • Pigmented hepatocellular adenoma (HCA) with beta-catenin activation have an increased risk for malignant transformation. • Surgical resection is the only curative treatment with best long-term outcome. • Molecular classification of HCAs is a useful tool for risk assessment of malignant transformation., Introduction Hepatocellular carcinomas (HCC) that are very large in size are a very rare finding in young adult. The malignant transformation of a pigmented hepatocellular adenoma (HCA) with beta-Catenin activation is a possible cause for appearance of HCC. Presentation of case We present the case of a 33-year-old male with a huge HCC with bone metastasis, emerged from pigmented HCA with beta-Catenin activation. As a two-stage surgical procedure, a left hepatectomy followed by a partial rib resection was performed. Discussion Giant hepatocellular carcinomas mostly develop in non-cirrhotic livers and at time of diagnosis an extrahepatic spread occurs in up to 15%. In the present case, the progression from a benign HCA to malignant HCC was documented, as a unique finding. Surgical resection is the only curative treatment and was successfully performed in this case. Conclusion Hepatobiliary surgery with resection of metastases is the treatment with best long-term survival for patients with huge HCC. Molecular characterization as well as pigmentation analysis is useful tools for risk assessment of HCA.