Juvenile spondyloarthropathies represent a clinical entity separate from the adult disease. Initial clinical signs of juvenile spondyloarthropathies often include lower extremity arthritis and enthesopathy, without axial involvement at the disease onset. Asymmetrical oligoarthritis of lower extremities is typically seen in this type of arthritis. Enthesopathy, which is the hallmark of the disease, is most commonly seen in the Achilles tendon, being manifested by heel pain. Anterior uveitis and HLA-B27 positivity are seen in a proportion of cases. Sacroiliitis is generally asymptomatic in the pediatric population. Ineffective treatment of childhood disease results in disease progression to typical adult form of ankylosing spondylitis. Therefore, early diagnosis and classification remains one of the most relevant questions in pediatric rheumatology. It should be kept in mind that the disease could be misdiagnosed as FMF or Behcet's syndrome in countries with a high incidence of those conditions. This review revises available classification criteria, clinical manifestations and therapeutic options for patients with juvenile spondyloarthropathies.