Background & Aim: Ataxia is a common presentation of various neurological disorders. It poses a challenge in the diagnosis of the cause and many patients remain undiagnosed. It is essential to understand the demographic pattern and clinical features for early diagnosis and effective management of the underlying disorder. Material and methods: This prospective, observational study was carried out at SCB Medical College, Cuttack, Odisha, India which is a tertiary care referral center, from October 2021 to September 2023. Detailed demographic, clinical, laboratory and electrophysiological data were collected from the case records of all patients presenting with subacute and chronic ataxia. Clinical history, physical examination findings, psychological and cognitive profiles were assessed in all cases. Routine blood investigations and electrophysiological evaluation including nerve conduction study was done in all cases whereas ophthalmological examination, neuroimaging, blood biomarkers estimation and nerve biopsy was done in selected cases. Somatosensory evoked potential, electroencephalogram, electrocardiogram, echocardiography, ultrasound abdomen and pelvis, chest X-ray was done in appropriate cases. Genetic testing was done in suspected cases of hereditary ataxia or sporadic spinocerebellar ataxias. Data were analysed using the SPSS statistical software. Data were expressed as mean and standard deviation for continuous variables and frequency (percentage) for categorical variables. Results:The hospital-based incidence of ataxia was 116 (4.9%). Out of 116 patients, 75 (64.6%) were males and 41 (35.4%) females respectively. Mean age of study population was 33.21 years. The mean age of disease onset was 33.1 yrs. The mean duration of disease was 2.08 years. Most common presenting symptom was motor weakness observed in 84 (72.4%) patients with ataxia. Other presenting symptoms were sensory(31%), vertigo(18.9%), convulsions(13.8%), visual abnormality(11.2%), ocular signs(13.8%), cranial neuropathies(14.6%), autonomic dysfunction(4.3%) and pseudo-dystonia(2.6%). Out of 116 patients, 71.5% presented with cerebellar ataxia whereas 28.4% had sensory ataxia. Amongst patients with cerebellar ataxia 32.5% had hereditary ataxia, followed by malignancy(16.9%), structural causes(14.4%), drugs(12%), degenerative(9.6%), demyelinating(6%), infection/post-infectious(4.8%), metabolic(2.4%) and mitochondrial(1.2%) causes . Sensory ataxia and Rhomberg’s sign were present in all patients of sensory ataxia. Diabetic polyradiculoneuropathy was a cause of sensory ataxia in 12.1% patients. Subacute combined degeneration of spinal cord was found in9.1% patients. A total of 78.8% patients had neuropathy and 21.2% pts. had myeloneuropathic sensory ataxia. Conclusion: Ataxia is a common manifestation encountered in various neurological conditions which can have acute, subacute, episodic or chronic presentation. It can be challenging to diagnose and may require urgent investigations such as brain imaging, genetic testing, immunological studies and blood investigations. The cause of ataxias should be identified early and the treatable conditions should be treated promptly with active monitoring.