OBJECTIVES: The article reports the case of encephalocele nasofrontal in an infant, relating to the genetic, syndromic and socioenvironmental factors, showing the importance of the health team being attentive to the findings, being a malformation, with a high mortality and morbidity rate and having few reports in the literature. CASE REPORT: Infant, female, born at term, vaginal delivery, 3.570g, natural and coming from Uberaba. After birth, she was hospitalized for a congenital heart disease and Down syndrome, cardiac surgery was performed. In this period, the respiratory physiotherapist perceived the difficulty of introducing the nasal aspirate, and was sent to the otorhinol, without statistical findings, a cranial tomography was performed at 4 months, which diagnosed an infant with nasoethmoidal and nasofrontal encephalocele. The encephalocele is a rare anomaly, with high morbidity, mortality and risk of complications. It is related to other pathologies diagnosed in patients and to the antecedent factors at birth, genetic and environmental causes. COMMENTS: Although it is difficult, with high mortality and morbidity, it is of great value to know about the factors that are related to this anomaly, both for the early diagnosis and for the prevention.