Background: Rare comprehensive epidemiological studies of ataxia have been undertaken worldwide. Objective: To estimate the prevalence of cerebellar ataxia and its subtypes in Al-Kharga District - New Valley. Methods: This is a community based study carried out through three stages. Total populations 62,583 were screened door to door (every door) by three specialists of neurology. All suspected patients were subjected to complete medical, neurological examination and brain MRI. Results: Twenty four patients of Cerebellar ataxia were recorded in this study with prevalence rate PR 38.34/100,000(95%CI; 23.01-53.69). The order of frequency of different types of cerebellar ataxia was as follows; 8 patients had vascular cerebellar ataxia (PR: 12.8/100000; 95%CI: 3.93 - 21.64), 6 patients had ataxic cerebral palsy (PR: 9.6/100,000; 95%CI: 1.92 - 17.26), 5 patients had unclassified progressive cerebellar ataxia (PR: 8/100,000; 95%CI: 0.99-14.99), 3 patients had post encephalitic ataxia (PR: 4.8/100000; 95%CI: 0.63 - 10.22) and 2 patients had Early onset hereditary Friedreich-like ataxia (PR: 3.2/100,000; 95%CI: 1.23 7.62). Conclusion: The prevalence rate of cerebellar ataxia in Al-Kharga District-New Valley (Egypt) is 38.34/100,000. [Egypt J Neurol Psychiat Neurosurg. 2010; 47(3): 527-532]