The association of celiac disease and idiopathic portal hypertension is a rare entity whose etiopathogenic mechanism has not yet been elucidated. We report two cases of celiac disease associated with idiopathic portal hypertension. In both cases celiac disease diagnosis was made based on the positivity of anti-tissue transglutaminase IgA and villous atrophy on the pathological study of duodenal biopsies. Idiopathic portal hypertension was retained after complete biological, radiological and endoscopic assessment excluding all possible causes that can manifest as portal hypertension. Both patients improved after gluten-free diet.