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Musculoskeletal anomalies are present in up to 50% of patients with prune belly syndrome (also known as Eagle-Barrett syndrome), requiring extensive orthopaedic management. However, to date, no established association has been reported between prune belly syndrome and autoimmune inflammatory arthritis. We present a case with prune belly syndrome who developed multiple joint pain and swelling. Initially, these symptoms were attributed to postural and structural abnormalities associated with the underlying condition. However, further evaluation led to a diagnosis of a seropositive polyarticular juvenile idiopathic arthritis. The patient was subsequently treated with a biologic agent, resulting in marked improvement in mobility and overall quality of life.