Abstract: STIL gene,an oncogene,was cloned from the T-cell acute lymphoblastic leukemia.STIL is closely related with the formation and duplication of centrioles and regulating cell mitosis in organism.Centriole is the center of cell mitosis.In cell mitosis,during the middle of G1 phase,STIL and PLK4(a centriole proliferation protein) form a complex containing coiled-coil domain (CCD) and STAN motif which participating the formation of centrioles and regulating cell mitosis.Besides,the number of the formation of centrioles is proportional to the content of STIL protein.Further studies indicate that in nervous system,STIL truncating mutations (STILT) mediate the development of MCPH (Microcephaly) by affecting the duplication of centrioles.STIL is also related to HPE (Holoprosencephaly).Enhancing the Shh signal transduction pathway mediate by STIL,which could make retinal dopaminergic cells reduce the sensitivity to neurotoxin,so as to protect the retina.It turns out that STIL expressed in multiple tumor tissues.STIL participates the formation,progress and metastasis of malignant tumor by regulating the number and structure of centriole,promoting the performance of chromosome instability.STIL is overexpressed in lung cancer especially in non-small cell lung cancer.In pancreatic cancer,STIL interacts with SUFU and Gli in Hedgehog pathway to mediating the progress of tumor.In this review,we aim to discuss the function of STIL in centriole and cell proliferation,elaborating it's association and potential mechanism in neurologic diseases and malignancies,providing a new direction of prognosis and treatment to the disease.