Abstract: Von-Hippel Lindau (VHL) syndrome is a rare clinical disease, and the cases with primary lesion located in the thoracic medulla are rarely seen. At present, surgery is still the main therapy for the VHL syndrome, and the therapeutic effect of microscopic minimally invasive surgery is better. In this paper, we retrospectively analyzed the clinical data of a case of VHL syndrome with thoracic medullary hemangioblastoma complicated with syringomyelia as the initial presentation, in order to provide reference for the diagnosis and treatment of this disease.