A 6-year-old girl with arthropathy accompanying sarcoidosis is presented. Articular, cutaneous and ocular features of sarcoidosis were prominent in this patient. Indirect immunofluorescence on HEp-2 cells revealed both antinuclear and anticytoplasmic antibodies. To determine distinguishing features between children with sarcoid arthritis and those with nonarticular sarcoidosis, reported cases from these 2 groups were reviewed. The mean age of onset of children with sarcoidosis and arthritis (2.3 years) was significantly lower than that of those with nonarticular sarcoidosis (10.8 years) (p less than 0.01). Skin was affected in 12 of 15 children with arthritis but in only 75 of 229 without arthritis (p less than 0.001). Ocular involvement was present in 14 of 15 patients with arthritis and in only 91 of 229 without arthritis (p less than 0.001). Among children with sarcoidosis the triad of arthritis, rash and uveitis occurs almost exclusively in those with an onset age of less than 5 years.