Abstract: Objective To investigate the value of magnetic resonance imaging in the diagnosis of Von Hippel-Lindau syndrome (VHL). Methods Clinical and multiple sites magnetic resonance data of 16 cases with VHL syndrome who were confirmed by pathology and imaging in Xinqiao Hospital of the Third Military Medical University from May 2010 to September 2015 were retrospectively analyzed, and the clinical and MRI findings were summarized to explore the ways of diagnosing this disease. Results Of the 16 cases, there were 10 females and 6 males with the mean age of 32.4 years.Ten patients had a family history of VHL. The MR data of 16 patients showed multi system disease, and all patients were complicated with hemangioblastomas in central nervous system and presented with "large cysts with mural nodule" mixed mass, including 5 cases of first-episode lesions; 6 cases had retinal hemangioblastoma, including 2 cases of first-episode lesions; 9 cases had renal cell carcinoma and renal cysts, including 4 cases of first-episode lesions; 7 cases had multiple pancreatic cysts, including 2 cases of first-episode lesions; 7 cases had pheochromocytoma, including 3 cases of first-episode lesions. There were also other patients presented with renal angiomyolipoma, liver hemangioma and so on. Combined the clinical and multiple sites MR data, the final diagnosis was 6 cases of type Ⅰ A, 3 cases of type Ⅰ B, 4 cases of type ⅡA and 3 cases of type Ⅱ B. Conclusion VHL syndrome has a genetic predisposition and often involves multiple organs. Hemangioblastomas in central nervous system and visceral lesions are the main first-episode lesions. Joint multiple sites MR is helpful for the diagnosis of VHL syndrome.