Sonic hedgehog (Shh) signaling pathway not only plays an important role in embryo development, but also participates in many physiological and pathophysiological processes. The pathological mechanism of the occurrence and development of ischemic stroke is complex, which has not been fully understood, and the current treatment is limited to an acute time window. As the role of Shh signaling pathway in ischemic stroke being increasingly reported, it was found that genes and proteins related to the pathway may participate in various stages of ischemic stroke and play different roles.This review summarizes main findings of the role played by Shh signaling pathway in the early stage, acute stage and recovery stage of ischemic stroke. It is expected that further study of Shh signaling pathway will help to provide valuable reference to new mechanisms and drug new target for the ischemic stroke.