An encephalocele results from failure of the surface ectoderm to separate from the neuroectoderm. The prevalance ranges from 0.8 to 4 per 10.000 live births. The occiput is the most common site. During a 3 years’ period 5 babies with occipital encephalocele were evaluated. All babies were girls. One mother was 45 years old, the others were around 25 years-old. All babies were born with cesarean sectio. Maternal folic acid (FA) consumption revealed that only 2 mothers used FA irregularly, not beginning preconceptionally. The rest 3 mothers did not use any supplementation nor any other drugs. One baby had also anencephaly and was lost on the 2nd day of life. The rest babies had hydrocephalus, corpus callosum agenesis, meningomyelocele and underwent surgery. One baby has serious feeding problems thus is on nasogastric feeding. The surviving babies are between 10–26 months, all having physical rehabilitation. Physically the 4 babies are below 3rd percentile both for weight and height. In conclusion, occipital encephalocele is a life-threatening cranial anomaly. The overall outcome of the patient depends on the site and dimension of the lesion, as well as presence of accompanying congenital anomalies. Close multidisciplinary follow-up is needed. FA supplemantation should be nationally provoked.