Neural tube defects (NTDs), including Spina bifida and hydrocephalus, are congenital birth defects of the nervous system which occur during early embryonic development. The worldwide incidence of NTDs is on average 1-2 per 1000 live births. NTDs have multifactorial etiology with associated effects of genetic variants and environmental factors. The registry of patients with NTDs was established in 1985 on the basis of the paediatric surgical department of Tallinn Children’s Hospital. The data from the registry allowed to present an overview of NTD patients in Estonia. In 2017 there were 127 alive patients with Spina bifida and 234 patients with hydrocephalus. The data for the past 30 years were used to calculate the birth prevalence of NTDs in Estonia. Data analysis, combined with genetic studies, has emphasized prophylactic consumption of folic acid. The data have also broadened our knowledge of the epidemiology of NTDs in Estonia and have addressed the need for improvement in the healthcare of young adult patients. It is also important to gain more thorough knowledge of NTDs from international studies.