Spinocerebellar degeneration with slow eye movements (SDSEM) is an extremely rare autosomal recessive disorder characterized by hereditary ataxia with slow eye movements. The condition has certain associated features, including progressive intellectual impairment, extra-pyrimidal dysfunction, peripheral neuropathy, and skeletal abnormalities. Although very similar to the Wadia Swami syndrome, SDSEM differs from the former in its relatively slow progression and recessive mode of inheritance.