Autosomal recessive cerebellar ataxias (ARCA) are a group of rare neurological disorders characterized by degeneration or abnormal development of the cerebellum and spinal cord, typically occurring before the age of 20 years. These disorders are inherited in an autosomal recessive manner and encompass a wide range of rare diseases, with Friedreich ataxia, ataxia-telangiectasia, and early-onset cerebellar ataxia with retained tendon reflexes being the most common. ARCA can be classified into five main types based on clinicogenetic criteria: congenital ataxias, ataxias associated with metabolic disorders, ataxias with DNA repair defects, degenerative ataxias, and ataxias associated with other features. Genetic mutations in specific genes, such as frataxin in Friedreich ataxia and α-tocopherol transfer protein in ataxia with vitamin E deficiency, have been identified. Clinical diagnosis is confirmed through neuroimaging, electrophysiological examinations, and mutation analysis. Treatment options are limited, with coenzyme Q10 deficiency and abetalipoproteinemia being the only conditions with specific treatments. Understanding the molecular mechanisms underlying these disorders is crucial for developing new treatments.Autosomal recessive cerebellar ataxias (ARCA) are a group of rare neurological disorders characterized by degeneration or abnormal development of the cerebellum and spinal cord, typically occurring before the age of 20 years. These disorders are inherited in an autosomal recessive manner and encompass a wide range of rare diseases, with Friedreich ataxia, ataxia-telangiectasia, and early-onset cerebellar ataxia with retained tendon reflexes being the most common. ARCA can be classified into five main types based on clinicogenetic criteria: congenital ataxias, ataxias associated with metabolic disorders, ataxias with DNA repair defects, degenerative ataxias, and ataxias associated with other features. Genetic mutations in specific genes, such as frataxin in Friedreich ataxia and α-tocopherol transfer protein in ataxia with vitamin E deficiency, have been identified. Clinical diagnosis is confirmed through neuroimaging, electrophysiological examinations, and mutation analysis. Treatment options are limited, with coenzyme Q10 deficiency and abetalipoproteinemia being the only conditions with specific treatments. Understanding the molecular mechanisms underlying these disorders is crucial for developing new treatments.