Metachromatic leucodystrophy (MLD) is a rare autosomal recessive, lysosomal storage disorder due to deficiency of enzyme arylsufatase A. It is classified clinically according to the age of onset into three forms: Late infantile, juvenile, and adult. MRI findings of MLD include symmetric confluent hyperintense areas in periventricular white matter with a demonstration of “tigroid” or “leopard skin” pattern in deep white matter. Prognosis in MLD is not good with progressive quadriplegia, decerebration, and they rarely live beyond mid adolocence invariably within 6 months to 4 years after onset. Our case is an illustration somewhat atypical presentation of MLD with monoperesis, with no intellectual dysfunction, incontinence, dysarthria and normal fundus.