While juvenile idiopathic arthritis is the most common inflammatory joint condition within the pediatric population, the burden of this pathology can be lessened by early detection and interdisciplinary management to avoid severe joint damage and skeletal deformity. The etiology of juvenile idiopathic arthritis is largely unknown; however, immunologic studies show possible involvement of CD141+, CD123+, and dendritic cells. The natural disease course consists of inflammatory soft tissue damage coupled with joint effusion that eventually progresses to bone and joint changes. In terms of diagnosis, musculoskeletal ultrasonography has been shown to be effective in the early detection of juvenile idiopathic arthritis, especially in small joints. Magnetic resonance imaging and radiography are also valid techniques for diagnosis, but they generally fail to detect preclinical juvenile idiopathic arthritis, whereas ultrasound is successful in doing so. Orthopedic treatment options include conservative measures such as non-steroidal inflammatory drugs and corticosteroid injections. Surgical intervention is often indicated to treat deformity, limb length, and severe arthritis. Total joint replacement is primarily performed for functional impairment and deformity rather than for pain. Factors that may complicate surgical intervention include small bone size, limb deformity, and soft tissue contracture.