Objective To analyze the clinical phenotypes of spinocerebellar ataxia type 2 (SCA2) in Xinjiang, China.Methods Physical examinations focusing on evaluating the nervous system were performed on patients considered as having SCA following Harding diagnostic criteria. The severity of ataxia and cognitive function were preliminarily evaluated by International Cooperative Ataxia Rating Scale (ICARS) and Mini-Mental State Examination. Genomic DNA was extracted by use of phenol-chloroform method and SCA subtypes of the participants were classified by PCR. Sequencing after cloning was conducted on one patient with SCA2.Results Nine patients with SCA2 were identified. The result of sequencing of the patient with SCA2 revealed CAG repeated 47 times. The patient with tremor onset showed clinical manifestations of Parkinson syndrome with a disease course of 6 years and an ICARS score of 10. Four patients had gait ataxia onset with a disease course of 2-4 years and an ICARS score of 15-26.Conclusions SCA can be preliminarily diagnosed by clinical manifestations. Because of the heterogeneity of clinical manifestations, the diagnosis of SCA2 is confirmed by gene diagnosis. Ataxia symptom developed slowly in patients who are diagnosed with SCA2 and mainly manifested by Parkinson syndrome.