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Göztepe Eğitim ve Araştırma Hastanesi, Nöroşirürji Bölümü, İstanbul, Türkiye Chiari malformations type 2 and type 3 are seen in children. Chiari malformation type 2 is often encountered in patients with spinal dysraphism whereas type 3 is a very rare condition. Both of these malformations are associated with multiple craniospinal anomalies. The most commonly encountered symptoms and signs are related to brainstem compression. Management should start with repair of the sac and treatment of the hydrocephalus if present. Foramen magnum decompression should be performed if brainstem compression symptoms occur despite these procedures. However, one must remember that these children have a high morbidity rate. Severe cognitive dysfunction can also be seen in children with Chiari malformation type 3. Anahtar Kelimeler : Chiari malformation, Child, Hydrocephalus, Spinal dysraphism