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¹Dr. Lütfi Kırdar Kartal Eğitim ve Araştırma Hastanesi, Beyin ve Sinir Cerrahisi Kliniği, İstanbul, Türkiye Hemangioblastomas of the central nervous system are benign tumors. They present sporadically or in association with von Hippel- Lindau disease. They are mostly located in the cerebellum, the brainstem, and the spinal cord. Atypical teratoid/rhabdoid tumor is a rare, extremely malignant embryonal tumor of the central nervous system, and occurs mostly in children under three years of age. Lhermitte-Duclos disease or dysplastic cerebellar gangliocytoma is a cerebellar disorder with features of cerebellar malformation, hamartoma, and benign neoplasma. These posterior fossa tumors are reviewed with their various characteristics. Anahtar Kelimeler : Hemangioblastoma, vonHippel-Lindau, Atypical teratoid/rhabdoid tumor, Lhermitte-Duclos, Dysplastic cerebellar gangliocytoma