We report the case of a 46-year-old man with von Hippel-Lindau (VHL) disease, manifesting disseminated leptomeningeal hemangioblastomatosis. The patient initially presented with a solitary hemangioblastoma in the right cerebellum. Later, he was diagnosed with VHL disease and underwent several surgical procedures in the following 14 years. But the prognosis was poor. Recently, the hemangioblastomatosis disseminated along leptomeninges involving both brain and spine. We aim to analyze the possible reason for the leptomeninges dissemination, discuss the imaging characteristics of this rare disease with ominous manifestation and propose the optimal strategy for treatment. We think the optimal treatment strategy should be surgical biopsy and surgical decompression. A long-term follow-up is inevitable. Antiangiogenic medication might be the hope for remission of this disease.