Autophagy is a critical process that maintains cellular homeostasis and integrity, particularly in the nervous system. The review covers the regulation of autophagy, from autophagosome biogenesis to lysosomal degradation, and its impact on neurodevelopmental and neurodegenerative disorders. It highlights the specific regulation of autophagy in neuronal compartments, such as the axon and somatodendritic regions, and how it is altered in diseases. The review also discusses the physiological functions of autophagy in the nervous system, including its role in development and adulthood. Key regulatory proteins and their mutations are discussed, along with the underlying pathophysiological mechanisms that contribute to neurodegenerative diseases. The compartmentalization of neuronal autophagy is explored, focusing on the biogenesis, maturation, and degradation of autophagosomes in different neuronal compartments. Finally, the review examines the implications of autophagy in neurological physiopathology, including its role in neural proliferation, differentiation, and synaptic function and plasticity.Autophagy is a critical process that maintains cellular homeostasis and integrity, particularly in the nervous system. The review covers the regulation of autophagy, from autophagosome biogenesis to lysosomal degradation, and its impact on neurodevelopmental and neurodegenerative disorders. It highlights the specific regulation of autophagy in neuronal compartments, such as the axon and somatodendritic regions, and how it is altered in diseases. The review also discusses the physiological functions of autophagy in the nervous system, including its role in development and adulthood. Key regulatory proteins and their mutations are discussed, along with the underlying pathophysiological mechanisms that contribute to neurodegenerative diseases. The compartmentalization of neuronal autophagy is explored, focusing on the biogenesis, maturation, and degradation of autophagosomes in different neuronal compartments. Finally, the review examines the implications of autophagy in neurological physiopathology, including its role in neural proliferation, differentiation, and synaptic function and plasticity.