Oz RS, et al. - Researchers reported a unique case of lipopolysaccharide (LPS)-responsive and beige like anchor (LRBA) deficiency first shown with polyarticular arthritis by conducting a literature review concentrating on LRBA deficiency, rheumatic disease, and arthritis in order to assess the features and peculiarities of arthritis in LRBA deficiency individuals. A total of 43 publications were analyzed. Out of this, seven individuals were recognized with arthritis. Two individuals were diagnosed with polyarticular juvenile idiopathic arthritis (JIA) and three with oligoarticular JIA. Each individual was discovered to have a distinctive genomic mutation. The treatment was different and included corticosteroids, cyclosporine, methotrexate, adalidumab, and abatacept. In summary, in LRBA deficiency, joint involvement was variable, therefore it shall always be kept in mind as a differential diagnosis for an individual with a combination of juvenile arthritis and clinically atypical immune dysregulation and/or immunodeficiency.