Urotensin Ⅱ is a kind of potent cyclic neuropeptide and has a series of biomedical functions. Recent studies have identified that the upregulation of urotensin Ⅱ and UT are involved in the process of organ fibrosis. The mechanisms of UⅡ effects are complicated and a number of signaling pathways activated by urotensin Ⅱ are related to myocardial fibrosis. This study reviewed the physiological and pathophysiological effects of urotensin Ⅱ involved in organ fibrosis, focusing on the correlation between urotensin Ⅱ and the concerned signaling pathways on myocardial fibrosis.