Budd-Chiari syndrome is a rather unusual clinical entity; among others, myeloproliferative disorders not infrequently are reported as a cause of this syndrome. In the past prognosis of Budd-Chiari syndrome was usually very poor. However, in recent years treatment with fibrinolytic agents has proved to be often successful in Budd-Chiari syndrome, as well as in other thrombotic disorders. In particular, r-TPA has appeared to be effective, due to its thrombospecificity. Three cases of Budd-Chiari's syndrome associated with myeloproliferative disorders are described, in which r-TPA administration, together with treatment of underlying disease, resulted in a complete recanalization of sovrahepatic veins. r-TPA, due to its thrombospecificity, has been shown to be more effective than other thrombolytic agents; its use is associated with a lower number of hemorrhagic events and it may be repeated in the case of uncompleted response.