Naturally occurring granular uranium particles are typically fine and are often found as accessory minerals. However, reports of megacrystalline uraninite are rare. The discovery of megacrystalline uraninite is a significant achievement in uranium prospecting and mineralogy in the Kangdian region and China. Our team’s research and review of previous studies have led to a systematic summary of the formation age, genetic types, relationship with migmatization, and metallogenic dynamic background of megacrystalline uraninite in the Kangdian region. The key findings are as follows: (1) the formation age of megacrystalline uraninite is Neoproterozoic (790–770 Ma); (2) migmatization preceded uranium mineralization; (3) the formation of megacrystalline uraninite is linked to high-temperature, low-pressure metamorphism caused by partial melting; (4) and the formation of megacrystalline uraninite may be associated with the Rodinia rifting event. This review aims to enhance our understanding of uranium mineralization during the Neoproterozoic in China and worldwide.