Spinocerebellar ataxia 3 is a rare hereditary neurodegenerative disease and the studies of the disease in Indonesia are still limited The disease initially focused on progressive motor disorders which are the main symptoms of SCA3 Along with the development of research SCA3 is also known can affect extracerebellar systems including cognitive function in the course of the disease The broad range of manifestations in SCA3 could be a challenge for its diagnosis and management Spinocerebellar ataxia 3 is a rare hereditary neurodegenerative disease and the studies of the disease in Indonesia are still limited. The disease initially focused on progressive motor disorders which are the main symptoms of SCA3. Along with the development of research, SCA3 is also known can affect extracerebellar systems including cognitive ...function in the course of the disease. The broad range of manifestations in SCA3 could be a challenge for its diagnosis and management.