Neuro-ophthalmological signs (N-OS) occur commonly in children with chronic ataxia. This study describes the N-OS and their frequencies, in general and by specific disease aetiology in paediatric patients with chronic ataxia. In total, 184 patients under the age of 17 years with chronic ataxia were identified. The study found 214 N-OS in 115 patients and the details of these signs along with patient diagnosis are recorded in detail by the authors. Strabismus was present in 29.3% of patients, nystagmus 27.7%, impaired smooth pursuit 23.4%, hypometric saccades 10.3%, decreased visual acuity 9.2%, abnormal optic discs 8.7%, abnormal pupillary examination 2.7%, hypermetric saccades 2.2%, impaired ductions 1.6% and abnormal visual fields in 1.1% of patients. Some N-OS were found more commonly in specific conditions, for example strabismus was most common following hypoxic-ischaemic encephalopathy after birth. The study findings are limited due to its retrospective nature. The study findings highlight the importance of a thorough examination of the visual and ocular motor systems. The findings from this ophthalmological assessment may aid in the assessment of these challenging children.