The juvenile-onset spondyloarthropathies are a group of pediatric disorders characterized by arthropathy and enthesopathy and a variety of extra-articular symptoms. With the application of new classification criteria, there is an increasing recognition of these diseases. This review summarizes recent advances in etiologic factors, clinical manifestations, therapeutics, and prognosis. Improved recognition of juvenile-onset spondyloarthropathies has allowed systematic and rigorous treatment trials to evaluate clinical outcomes relevant to pediatric medicine. Thus far, current therapeutic options allow for symptomatic control only. Further treatment studies are needed to examine the possibility of disease modification of juvenile-onset spondyloarthropathies.