Hemangioblastoma is a relatively rare benign tumor of the central nervous system. It can occur sporadically or as part of von Hippel-Lindau (VHL) disease,accounting for approximately 2% of intracranial tumors and 2%~10% of primary spinal cord tumors. Imaging examinations are important means for preoperative diagnosis of hemangioblastoma,while the final diagnosis relies on pathological diagnosis. Currently,the main treatment for hemangioblastoma is microsurgery,which has a low mortality rate and favorable long-term surgical outcomes. In recent years,with in-depth research,there have been new insights into hemangioblastoma in clinical practice. This article presents a review of the progress in the diagnosis and treatment of hemangioblastoma of the central nervous system,providing a reference for the clinical diagnosis and treatment of hemangioblastoma.