We describe for the first time a case of idiopathic portal hypertension (IPH) in a 31 year old patient with coeliac disease (CD). She had splenomegaly, pancytopenia and an unexplained portal hypertension in the absence of any histological abnormalities of the liver. The diagnosis of coeliac disease was based on histological and serological data. Treatment included a splenectomy and a splenorenal shunt.