Peptide urotensin II was originally isolated from the urophysis of teleost fishes; later it was identified also in higher vertebrates in various organs and tissues, including cardiovascular structures. Since its discovery it has been considered as a highly potent vasoconstrictor inducing contraction of smooth muscle in subnanomolar concentrations. Its wide distribution as well as its high interspecies homology indicates that this peptide is involved in regulation of many important physiological functions in vertebrates. An effort to discover other possible functions of urotensin II was intensified by the identification of its G-protein coupled receptor and its identification in humans. Furthermore, altered levels of expression of urotensin II and its receptor were found in various disease states including hypertension, diabetes, heart and renal failure, in experimental animal models as well as in humans. Therefore, there is widely discussed question regarding the possible role of urotensin II in etiopathogeneses of these diseases, however the exact mechanisms are still unknown. The aim of this review is to summarize the current knowledge about urotensin II with emphasis to its direct and undirect effects in cardiovascular system.