Abstract: Immunodeficient animal models are generated for biomedical research by spontaneous mutations or genome editing to remove one or more components of the immune system. Immunodeficient mice models have been well established, which provide good tools for many research areas such as humanize mice, patient-derived xenograft (PDX), stem cell transplantation and infectious diseases. Compared with mice, however, rats have the advantages of large size, more similar to human metabolically and  physiologically, easier surgical operation and sample collection, and long-term applicated in preclinical toxicology and pharmacology. Therefore, in recent years, a variety of immunodeficient rat models have emerged. By comparing different animal models, this review mainly focuses on the recent advances in immunodeficient rat models, and discusses their potential applications in stem cell and antitumor therapy.