Background: Dopa-responsive dystonia (DRD) is a rare disorder with a classic presentation of childhood or adolescent-onset dystonia. This is characterized by parkinsonism, diurnal fluctuations, and a dramatic response to low doses of levodopa. It has been reported that female carriers of the genetic mutation are more often affected than males. Methods: A 62-year-old man with DRD. He is a sibling of the first documented case of DRD with autopsy. Results: He noticed mild clumsiness at 10 years old when he would fall playing games. He gave up playing hockey in his 30s because of his balance. Neurological exam at age 49 revealed mild findings of parkinsonism and dystonia of the right leg. He was started on levodopa/carbidopa 100/25 mg 1/2 pill BID with significant and sustained improvement and resumed playing hockey. His sister had onset at age 5 with walking on tiptoes with obvious dystonia when examined at age 8; she was well controlled for many years on straight levodopa (without decarboxylase inhibitor) at a dose of 250mg TID. Conclusions: These cases of siblings with DRD exemplify varying degrees of severity among family members and genders with the same condition. Genetic results are pending and will be presented.