A 43-year-old male with a history of congenital hepatic fibrosis associated with large liver cell dysplasia developed a sizable lesion in the right lobe of the liver, which was, after a follow-up of 4 years, surgically removed on account of a suspected malignant transformation. Pathological examination showed an unencapsulated nodule with both architectural and cytological dysplastic changes, arising in a background of congenital hepatic fibrosis harbouring extranodular foci of large liver cell dysplasia. This report concerns the development of a high grade dysplastic nodule, a sizable hepatocellular lesion with suspected preneoplastic significance, in a patient with congenital hepatic fibrosis associated with large liver cell dysplasia.