Background　Paired-box 6 （Pax6） is a highly conserved transcription member of homebox family with numerous actions in forebrain development， while missing or mutations of Pax6 can lead to abnormal structure and function of brain.　Objective　To review the role of Pax6 in forebrain development and cognitive function.　Content　In this literature， we will introduce the characteristics of Pax6， and the role of Pax6 in forebrain development， especially in the correct specification of neural progenitors， the balance between proliferation and differentiation of neural progenitor cells and the regulation of cortical neuronal migration. Moreover， we will also introduce the Pax6 related cognitive dysfunction in this review.　Trend　Further researches into this area may provide useful insights and directions into the Pax6 related cognitive dysfunction.