Spondyloarthropathy (or spondyloarthritis) can begin in childhood (defined as individuals less than 16 years of age). These diseases are distinct in childhood, when compared with adult-onset disease. Because of overlapping features, especially sacroiliac joint involvement, diagnostic difficulty may arise from Behcet's disease, as well as familial Mediterranean fever. Despite advances in diagnostic techniques such as magnetic resonance imaging, the diagnosis of juvenile spondyloarthropathy may still be delayed many years from the onset of symptoms. Treatment of juvenile spondyloarthropathy has advanced rapidly in the last several years, with increasing evidence that agents targeting tumor necrosis factor are effective. These agents also have serious complications, including induction of other autoimmune diseases.