A 73-year-old woman was admitted after the first upper gastric tract haemorrhage due to gastric variceal bleeding. A CREST syndrome associated with Hashimoto's thyroiditis, Gougerot-Sjögren syndrome, cryoglobulinaemia and complicated with severe pulmonary hypertension was diagnosed. Liver histology found precirrhotic lesions of primary biliary cirrhosis (PBC) and nodular regenerative hyperplasia (NRH). Collagen diseases are often associated with liver test abnormalities and liver disease usually associated with CREST syndrome is PBC. NRH has been found in association with collagen diseases but also with haematological diseases or drugs or with autoimmune diseases, such as PBC. This case shows the association of PBC and NRH with porto pulmonary hypertension in CREST syndrome.