Mitochondria are ubiquitous and multi-functional organelles involved in diverse metabolic processes, namely energy production and biomolecule synthesis. The intracellular mitochondria! morphology and distribution change dynamically, which reflect the metabolic state of a given cell type. A dramatic change of the mitochondria! dynamics has been observed in early development that led to further investigations on the relationship between mitochondria and the process of development A significant developmental process to focus on, in this review, is a differentiation of neural progenitor cells into neurons. Information on how mitochondria-regulated cellular energetics is linked to neuronal development will be discussed, followed by functions of mitochondria and associated diseases in neuronal development Lastly, the potential use of mitochondria! features in analyzing various neurodevelopmental diseases will be addressed.