Hepatocellular carcinoma (HCC) is primarily a malignant liver tumour. In rare cases, HCC may be asymptomatic and incidentally identified in radiological exams. Although extrahepatic metastasis to organs such as the lungs or bones is not very uncommon, isolated extrahepatic lesions with hepatoid characteristics, in the absence of a primary tumour in the liver or gastrointestinal tract, are incredibly uncommon. Herein, we present an intriguing case of an isolated skull lesion with hepatoid features with no identifiable primary hepatic or gastrointestinal lesion. This case highlights the need for further research into these rare presentations to deepen our understanding of HCC pathogenesis and ectopic tissue transformation.