The Chiari malformations (CM) are herniations of the hindbrain structures through the foramen magnum. According to the original classification, there are four types. Magnetic resonance imaging (MRI) is the gold standard for diagnosis. CM Type I is the most common formation in childhood. With the widespread of MRI scans, they are commonly observed radiologically, but only some cases present clinical symptoms. Syringomyelia is the most commonly associated clinical condition; when detected, it can remain asymptomatic for a long time and is also indicative for treatment. CM type II is the advanced form of CM I accompanied by brainstem dysfunction and is almost always observed with myelomeningocele and hydrocephalus.