Leucine rich-repeat sequence (LRR sequence) is a special class of polypeptide sequences composed of several adjacent leucine residues. LRR sequence participates in evolution between species and across species. This sequence is widespread in a variety of proteins and has important biological functions. LRR protein plays a major part in the development of the nervous system and the maintenance of normal function. In addition, a large number of leucine repeats are closely related to the occurrence of a variety of diseases, such as leucine-rich repeat disease, leucine-rich peptide aggregate. Most importantly, it correlates with the development of brain tumors like glioma. This article is primarily focused on the structure and function of LRR sequence and the associated LRR proteins. Moreover, we summary the LRR-contain transmembrane proteins in nerve system and highlight the function of these proteins in glioma progression which takes the sequencing of LRR a potential personalized diagnosis and treatment of brain tumors.