Uveitis is a severe manifestation of a systemic connective tissue disease in both adults and children. Early identification of children at risk of developing this disease and adequate treatment can save the child’s vision. The optimal treatment for such children is a multidisciplinary approach in collaboration with a pediatric rheumatologist and an ophthalmologist. This review provides information about risk factors for the development and severe course of uveitis, taking into account clinical and genetic components. Data on the treatment of the disease, taking into account modern pathogenetic approaches using genetically engineered biological therapy are given.