Only few case reports of SCA7 have been reported  in India. one case of  SCA7 was reported  by Mittal et al (2005)without family history,which was later found to be postzygotic denovo expansion  of 59 CAG repeats on paternal allele of the ATXN7 gene(2). Another case of SCA7 reported from ethnic tribe of Assam(Bodo community)  with history of similar symptoms in mother and maternal uncle(3).Recently an Indian family is reported from  Belguam region of Northern Karnataka in South India .

We are presenting a case of SCA7 in Indian family from Ismailpur village ,Alwar district, Rajasthan.This is the first case reported from North –West India to our best knowledge .