Since 1990, altogether 16 INCL patients received lamotrigine (LTG) because of intractable epilepsy. The response to LTG was favorable in 15/16 children. The severity of seizures decreased significantly in 15/16 patients, the frequency of seizures decreased in 14/16, and the effects were maintained. In addition, LTG had a beneficial effect on the well-being of 14/16 children. LTG failed to maintain it's efficacy in monotherapy. No severe side effects were found.