A retrospective study was carried out in Oman to determine the incidence of neural tube defects (NTD) and congenital hydrocephalus (CH) and to identify any possible associations. National data retrieved from hospital records revealed the incidence of NTD in Oman to be comparatively low (1.25 per 1000), but the incidence of CH was much higher than that seen in Western Europe (0.44 per 1000) and was found to be associated with high rates of other congenital anomalies and neonatal death. There were no specific environmental factors associated with NTD and high environmental temperatures during the tropical desert summer (temperatures reach 48°C) were excluded as a causative factor. In spina bifida families, later born children were more likely to be affected and there was also an association with increased maternal but not paternal age. Much higher consanguinity rates were noted in families with NTD and CH than in the general population.