Abstract: Magnetic resonance imaging (MRI) is an important imaging modality for clinical disease diagnosis, and nearly 50% of clinical MRI examinations require contrast agents to enhance the diagnostic sensitivity. This review provides a summary of the major MRI contrast agents and their classification, and the advantages and limits of the commercially available MRI contrast agents, and elaborates on the exceedingly small magnetic iron oxide nanoparticles (ES-MIONs), dotted core-shell iron and gadolinium hybrid nanoparticles (FeGd-HN) and exceedingly small gadolinium oxide nanoparticles (ES-GON). These nanoparticles can greatly improve the efficiency of T1-weighted MRI due to their high r1 value and low r2/r1 ratio, and are expected to be translated into clinical contrast agents for T1-weighted MRI. The authors also review the diagnostic and therapeutic integration system that combines MRI contrast agents with various tumor therapies, such as MRI-guided ferroptosis therapy, radiosensitization therapy, and photothermal therapy, which allow efficient treatment as well as real-time monitoring of tumors and serve as potential cancer therapy strategies. The possible future research directions in the field of MRI-based multifunctional diagnostic and therapeutic formulations are also discussed.