Celiac disease can present great clinical heterogeneity. Its association with a series of intestinal and non-intestinal diseases, whether immunologically mediated or otherwise, presents a higher than normal frequency. We present a patient with celiac disease and Budd-Chiari syndrome of unknown cause. This association has previously been described only in isolated cases in northern Africa. The appearance of this case in Spain reveals that the coexistence of both processes in a single patient is unlikely to be due to environmental or geographical factors.