Eight children with pyridoxine-dependent seizures (PDS) were seen over a period of 10 years. Of those children, 6 are on regular follow-up. Four of the children were seen in one family. All the patients presented with refractory seizures, mainly neonatal status epilepticus. Though PDS is a rare condition, it must be considered in all cases with refractory seizures, particularly in childrens younger than 3 years. When confirming a diagnosis, oral pyridoxine is as effective as intravenous pyridoxine.