Nipah virus (NiV), an emerging zoonotic virus, has been associated with several outbreaks with high death rates, mainly in South and Southeast Asia. NiV is responsible for Encephalitis and systemic vasculitis, and occasionally respiratory diseases accompanied by it. Though fruit bats are the natural source of NiV, it can be transmitted in a zoonotic manner directly or via an intermediate host (e.g., a pig or horse). Several studies explore the viral mechanism of disease progressions and its overall pathogenesis. However, understanding the pathogenesis and disease dynamics is necessary to develop therapeutic options and vaccines. Thus, in this review, we provide a comprehensive update on the emerging understanding of the pathogenesis of NiV.