Von Hippel-Lindau disease is a rare disorder which involves multi-organs including cerebellum and retina. Recently we have experienced 2 cases of von Hippel-Lindau disease. The case 1 was a 19-year-old female with chief complaint of severe headache accompanied by vomiting on admission. Computed tomogram of brain showed space occupying lesion in the posterior fossa. She was rapidly deteriorated and diagnosed as brain death on the basis of physical and neurological examination and electroencephalogram. Kidney specimens removed for the transplantation and cerebellum obtained by partial autopsy reveled bilateral renal cell carcinomas and hemangioblastoma, respectively, on the microscopic examination.. The case 2 was a 23-year-old male who visited to our hospital due to headache, gait disturbance, and visual loss in the right eye. The findings of brain CT, vertebral angiography, ophthalmoscope, retinal angiogram, and operation were compatable to cerebello-retinal angiomatosis. With reviewing of some literatures, we present 2 cases of von Hippel-Lindau disease.