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¹Beyoğlu Göz Eğitim ve Araştırma Hastanesi, 2. Göz Kliniği İstanbul, Uz. Dr.
²Beyoğlu Göz Eğitim ve Araştırma Hastanesi, 2. Göz Kliniği İstanbul, Doç. Dr. Von Hippel-Lindau (VHL) disease is an inherited multisystem cancer syndrome caused by mutations of the VHL gene on chromosome 3p25. The prevalence of disease ranges from 1/30.000-50.000. The neurological complications arising from cerebellar hemangioblastomas or metastatic renal cell carcinomas are the most common cause of death in VHL patients. The patient with VHL disease who just admitted with complaints of bilateral visual loss was presented in this paper. Keywords : Von Hippel-Lindau disease, retina and cerebellar hemangioblastoma