Objective To summarize the imaging findings of intracranial central nervous system(CNS)malformations children in the high-altitude plateau(Xigaze city).Methods We retrospectively reviewed the imaging data of the suspected patients having CNS malformations,which enrolled either by the clinic or by the ultrasound in the Shigatse People's hospital from June,2019 to June,2023.All imaging data were interpreted by two experienced radiologists with consensus reading.Imaging characteristics were recorded roughly by primary/secondary,supra-/subtentorial malformation type and the corresponding clinical symptoms.Results A total of 36 children were included in this study,including 19 patients≤1 year old(including 2 newborns),7 patients between 1 and 3 years old,and 10 patients>3 years old.Seven cases underwent MRI examination,others having CT scan.Polygyria and pachygyria malformation were the most common type of congenital neurological malformations(7 cases,31.8%),followed by cystic changes of the cerebral parenchyma(3 cases,13.6%).Cerebral atrophy was the most common type of secondary CNS abnormalities group(8 cases,57.1%),followed by communicative hydrocephalus(3 cases,21.4%).Five patients in the congenital group and 4 patients in the secondary CNS group had the complex malformations.There were 8 dead cases(all in the≤1-year-old group),12 cases having neurological sequelae,1 case with normal development and 15 cases with loss of follow-up.There were no significant differences between primary and secondary CNS group regarding the final outcome.Conclusion In this study,gyrus developmental malformation and encephalatrophy were the most common etiologies in infants'CNS malformations.Reasonable use of imaging modalities will help depict the complicated CNS malformations and design individual treatment.