Autosomal recessive cerebellar ataxias (ARCA) are a group of rare neurological disorders characterized by cerebellar and spinal cord degeneration, autosomal recessive inheritance, and early onset before age 20. They include several rare diseases, such as Friedreich ataxia (prevalence 2–4/100,000), ataxia-telangiectasia (1–2.5/100,000), and early-onset cerebellar ataxia with retained tendon reflexes (1/100,000). ARCA can be classified into five main types: congenital ataxias, metabolic ataxias, DNA repair defects, degenerative ataxias, and ataxias with additional features. These disorders are caused by mutations in specific genes, such as frataxin in Friedreich ataxia, α-tocopherol transfer protein in ataxia with vitamin E deficiency (AVED), aprataxin in ataxia with oculomotor apraxia (AOAI), and senataxin in ataxia with oculomotor apraxia (AOA2). Diagnosis involves clinical evaluation, neuroimaging, electrophysiological tests, and mutation analysis. Genetic counseling is crucial for prognosis and treatment. ARCA is typically autosomal recessive, so family history is unlikely. Treatment options are limited, with coenzyme Q10 deficiency and abetalipoproteinemia being exceptions. The article discusses various ARCA subtypes, their genetic basis, clinical features, and diagnostic methods. It also covers the pathogenesis of ARCA, including the role of frataxin in mitochondrial function and iron homeostasis, and explores therapeutic approaches such as antioxidants and idebenone. The review highlights the importance of molecular diagnosis in understanding and managing ARCA.Autosomal recessive cerebellar ataxias (ARCA) are a group of rare neurological disorders characterized by cerebellar and spinal cord degeneration, autosomal recessive inheritance, and early onset before age 20. They include several rare diseases, such as Friedreich ataxia (prevalence 2–4/100,000), ataxia-telangiectasia (1–2.5/100,000), and early-onset cerebellar ataxia with retained tendon reflexes (1/100,000). ARCA can be classified into five main types: congenital ataxias, metabolic ataxias, DNA repair defects, degenerative ataxias, and ataxias with additional features. These disorders are caused by mutations in specific genes, such as frataxin in Friedreich ataxia, α-tocopherol transfer protein in ataxia with vitamin E deficiency (AVED), aprataxin in ataxia with oculomotor apraxia (AOAI), and senataxin in ataxia with oculomotor apraxia (AOA2). Diagnosis involves clinical evaluation, neuroimaging, electrophysiological tests, and mutation analysis. Genetic counseling is crucial for prognosis and treatment. ARCA is typically autosomal recessive, so family history is unlikely. Treatment options are limited, with coenzyme Q10 deficiency and abetalipoproteinemia being exceptions. The article discusses various ARCA subtypes, their genetic basis, clinical features, and diagnostic methods. It also covers the pathogenesis of ARCA, including the role of frataxin in mitochondrial function and iron homeostasis, and explores therapeutic approaches such as antioxidants and idebenone. The review highlights the importance of molecular diagnosis in understanding and managing ARCA.