Juvenile spondyloarthritis (SpA) is a distinct form of juvenile arthritis characterized by male predominance and adolescent onset. Clinical manifestations include lower extremity and sacroiliac joint arthritis, enthesitis, and subclinical gastrointestinal inflammation. Juvenile SpA is an immune-mediated inflammatory disease long recognized as associated with HLA-B27, which may be related to the microbial environment as suggested by its coexistence with reactive arthritis and psoriasis. Treatment of peripheral arthritis includes nonsteroidal anti-inflammatory drugs, joint injections, and disease-modifying agents, whereas treatment of axial disease may necessitate a tumor necrosis factor inhibitor biologic agent. Fewer than half of children achieve remission off medication 5 years after diagnosis.