Juvenile idiopathic arthritis-associated uveitis (JIA-U) is a prevalent non-infectious autoimmune uveitis among children under the age of 16. The principal pathogenesis lies in the interaction between genetic and environmental factors, resulting in autoimmune dysregulation within the body. Under the collective influence of these factors, the autoimmune response in JIA patients may disrupt peripheral immune tolerance and the blood-retinal barrier, leading to the infiltration of T and B cells and their subsets into the ocular tissues of patients. Through reacting to specific retinal antigens, the production of highly specific autoantibodies, and secreting cytokines such as tumor necrosis factor α (TNF-α) and interleukin 6 (IL-6), it promotes the occurrence and progression of ocular tissue inflammation, and in severe cases, poses a threat to the patient's vision. The initial treatment for JIA-U is based on a stepwise strategy, with corticosteroids as the first-line drug, combined with methotrexate (MTX) for steroid-reduced systemic therapy. For patients intolerant to MTX or with suboptimal therapeutic responses, or those presenting with severe vision-threatening complications, biological disease-modifying antirheumatic drugs (bDMARDs) can be added or substituted. TNF-α inhibitors, IL-6 receptor inhibitors, and Janus kinase inhibitors are commonly utilized bDMARDs for refractory JIA-U or patients unresponsive to MTX. Additionally, T-cell co-stimulation modulators and anti-CD20 monoclonal antibodies may also prove effective for refractory JIA-U or patients unresponsive to MTX. Future studies are requisite to further investigate the mechanism of action, safety, and efficacy of these biological agents in JIA-U, in order to provide more effective therapeutic references.