Introduction: The Central Nervous System (CNS) affects 5-10 children per 1,000 newborns. The high prevalence is for the reason that CNS could be more affected than others organs because your long and complex development, which reflects an increased illness and mortality of that children. Objective: Assess the clinics and epidemiologic aspects of CNS malformations and comparing to the anatomical pattern which occur in children and teenagers taken care in two medical centers at Belém, Pará. Methodology: A study of 239 children and teenagers with CNS malformation diagnosed taken care in neuropediatrics clinic of Hospital Universitário João de Barros Barreto e Hospital Ofir Loyola in Belém, Pará, since January 2005 until March 2008. Results: The patients were male (61.1%), infant (35.0%), no CNS malformation or psychiatric disease familiar history (66.9%). The Associated Malformation was the most important like group (28.0%) and hydrocephalus as specified disease (18.4%) at study. The main sings and symptoms were seizures (64.9%), neuropsychomotor development delay (52.7%), motor deficit (39.3%), learning deficit (25.5%) and sensorial-sensibility deficit (4.2%). All the groups showed abortion report before the patient’s pregnancy, that match 22.2% (p = 0.0223). The parents were consanguineous in four cases. Most of the mothers did prenatal (64.9%). It was observed that 6.1% smoked (p = 0.0135), 7.9% drank, 1.2% was addicted, 20.1% used some prohibited medicine in pregnancy, 14.2% reported infectious disease and 31.0% prenatal complications. The newborn did natal and postnatal complications in 9.2% and 18.8% of the cases, respectively. Vaginal way was done in 60.2%. Most of newborn born between 37-42 pregnancy weeks (82.2%) (p = 0.0045) and the weight average at born was about 2,903g. The skull magnetic resonance was requested in 73.2% and medicine’s was prescribed in 85.8%. Final Considerations: The congenital malformation earn a big repercussion in public health getting multicenter study to search better risk factors, signs and symptoms as a intention to up grade the life quality of patients.
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