Childhood hereditary ataxias are a degenerative and extremely heterogeneous group. They are classified in various ways in the literature (1-3). While a rare group may have a possibility of treatment, specific diagnosis is especially important for genetic counseling. 196 case files suggesting cerebellar ataxia findings were selected from 30,000 patients admitted to our clinic between September 2010 and February 2013. Files included in this study were evaluated according to age, sex, clinical characteristics, patient and familial history, head circumference, cognitive capabilities and other neurological findings, labaratuvary findings and cranial imaging findings. Based on the recorded information, and undiagnosed group and the diagnosed group were established, referred to as groups I and II respectively. Group I  and Group II  consisted of 157 (81.1 %) and 39 (19.9 %) patients, respectively. Group II differed from group I in terms of absence of deep tendon reflexes, polyneuropathic changes at electromyelography , pathological visual evoke potential , electroretinogram and normal mental and motor development milestones before diagnosis (p< 0.005).  In contrast, the presence of cerebellar atrophy and/or other abnormalities in MRI finding made diagnosis more diffucult (p <0.01). Accompanying epilepsy and mental retardation did not lead to a difference between the groups. There was also no difference between the groups in terms of sex, familyal history, mental retardation, vitamin B12 and E, epilepsy, basal methabolic tests, organomegaly or brain auditory evoked response (BAER) (P>0.05). The presence of neuropathy, VEP, ERG abnormalities, normal mental motor developmental milestones before the diagnosis and consanguinity were the factors that facilited the diagnosis.