Autoimmune diseases are diseases characterized by local or systemic abnormal inflammatory immune responses. With the in-depth exploration of the pathological mechanism of autoimmune diseases, it is found that occurrence and development of autoimmune diseases are largely related to the interaction between chemokine receptors and chemokines expressed at inflammatory sites. CCR7, one of Chemokine receptors members, binds to CCL21, which regulates lymphocyte homing, neovascularization and immune cells migration in autoimmune diseases. However, the underlying signaling pathways of CCL21/CCR7 need to be further explored. Despite the enormous advances in our knowledge of chemokines, research about the involvement of CCL21/CCR7 in autoimmune diseases progression is still limited. Thus, in this review, we summarize the essential role of CCL21/CCR7 in autoimmune diseases progression. Further studies are critical to illustrate the distinct roles of CCL21/CCR7 in autoimmune diseases progression, and are important significance for discovery of new biomarkers and drug targets of autoimmune diseases.