Ribbon like heterotopia is a unique brain malformation and recently discovered novel entity caused due to loss of function or missense mutation involving EML 1 gene (Echinoderm microtubule-associated protein-like 1). Periventricular and ribbon like subcortical heterotopia, megalencephaly, complete or partial callosal agenesis and polymicrogyria like “lumpy-bumpy” appearance of the overlying cortex are some of the distinctive features elaborated in the literature.