News. Craniocerebral malformations (CCM) are of major importance for psychomotor retardation and seizures in children. Mortality is high, estimated at 40% of deaths in the first year of life. Aim of the study. To estimate the clinical and imaging manifestations of MCC in children in order to approach early diagnosis and recommend prophylactic methods. Materials and methods. Eighteen children, aged 1 month to 3 years, detected with various types of MCC were evaluated. Neurological examination - by Amiel-Tison method and imaging by CT and/or brain MRI. Statistical methods used: t-student test and 95CI confidence coefficient. Results. Of the 18 children with MCC - 12 (66.7%; 95CI 55.59-77.81) were detected in the first year of life by imaging examination. Types of MCC: neurolational and neural tube formation disorders (5.6%), brainstem anomalies (5.6%), ventral induction anomalies (5.6%), cerebellar malformations (11.1%), Dandy Walker anomaly (11.1%), congenital hydrocephalus (16.7%), anomalies associated with disorders of cortical development (22.2%), corpus callosum agenesis (22.2),. Common clinical manifestations: developmental delay, axial and limb hypotonia, seizures, spastic hemi/tetraplegia, microcephaly, hemianopsia, ataxia. Conclusions: MCC presents with polymorphic clinical manifestations, ranging from mild to severe, sometimes incompatible with life. Diagnosis of the MCC type is made by imaging examinations. The most common MCCs detected: agenesis of corpus callosum and cortical developmental disorders Dandy Walker anomaly. MCC prophylaxis is necessary in the antenatal period.