Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare genetic disorder, inherited as an autosomal recessive or autosomal dominant pattern. It predominantly involves white matter of the brain. Megalencephaly is the most common and earliest clinical finding during infancy, which is followed by progressive pyramidal signs, ataxia, cognitive impairment and seizures in some patients. Magnetic resonance imaging (MRI) brain shows predominant frontotemporal white matter involvement with subcortical cysts. Though the majority of the described cases of MLC in India belongs to the Agarwal community, our patient is non–Agarwal Hindu.