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 lo
be of the liver which was, after a follow-up of 4 years, surgically removed
on account of a suspected malignant transformation. Pathological examinati
on 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 con
cerns the development of a high grade dysplastic nodule, a sizable hepatoce
llular lesion with suspected preneoplastic significance, in a patient with
congenital hepatic fibrosis associated with large liver cell dysplasia.