Periventricular Nodular Heterotopia develop as a consequence of non -migration and developmental arrest of neurons along the ependymal -subependymal margins of lateral ventricles. The frontal predominant periventricular nodular heterotopia is X-linked and associated with FLNA mutation (Filamin A gene) and found in females. Posterior periventricular nodular heterotopia is a distinct entity ( FLNA -ve).Cortical and posterior fossa malformations, callosal anomalies , white matter volume loss , colpocephaly and ventriculomegaly are frequently observed.