A unique case of Ito syndrome presenting with Juvenile Myoclonic Epilepsy and normal intelligence

Background: Hypomelanosis of Ito is a neurocutaineous disease commonly associated with seizure and mental retardation. Case description: In this case report, we have found a unique coexistence of Hypomelanosis of Ito and Juvenile Myoclonic Epilepsy with normal intelligence. Discussion: The occurances of two genetically linked disease entities in a single individual arouse a possibility of some common genetic factors. Conclusion: The Juvenile myoclonic Epilepsy with normal intelligence can be a presenting feature in Hypomelanosis of ito. Key message: In contrary to common perception Hypomelanosis of ito can present with normal intelligence and the seizure type can be Juvenile myoclonic epilepsy as found in this case