Hippo signaling pathway is a highly conserved and crucial signaling pathway that controls the size of tissues and organs by regulating the proliferation, differentiation, and apoptosis of cells. The nervous system is a complicated system that participates in information collection, integration, and procession. The balance of various aspects of the nervous system is vital for the normal regulation of physiological conditions of the body, like the population and distribution of nerve cells, nerve connections, and so on. Defects in these aspects may lead to cognitive, behavioral, and neurological dysfunction, resulting in various nervous system diseases. Recently, accumulating evidence proposes that Hippo pathway maintains numerous biological functions in the nervous system development, including modulating the proliferation and differentiation of nerve cells and promoting the development of synapse, corpus callosum, and cortex. In this review, we will summarize recent findings of Hippo pathway in the nervous system to improve our understanding on its function and to provide potential therapeutic strategies of nervous system diseases in the future.