Background: Sarcoidosis is a rare cause of uveitis in childhood. Differentiation from other rheumatic diseases may be difficult. We report on five children with uveitis in childhood sarcoidosis and review the previous literature. Results: Children were aged between seven month and 13 years at the time of diagnosis of uveitis. Four of the five patients had panuveitis, the fifth one had intermediate uveitis. In addition, three children had systemic diseasewith skin, joint and central nervous system involvement. The two children younger than 5 years showed the clinical triad of eye, joint and skin disease. Uveitic complications included corneal band keratopathy, cataract, posterior synechiae, macular scars and choroidal neovascularisation. Vision-limiting complications were found in six of nine eyes at the first visit. Conclusion: In childhood sarcoidosis two clinical courses can be differentiated. Younger children aged up to five years express a clinical triad of arthritis, skin lesions and uveitis. In the older group of patients, the clinical course is more similar to that of adults. Rapid diagnosis and quiescence of disease are of particular importance to improve the long-term visual prognosis. Background: Sarcoidosis is a rare cause of uveitis in childhood. Differentiation from other rheumatic diseases may be difficult. We report on five children with uveitis in childhood sarcoidosis and review the previous literature. Results: Children were aged between seven months and 13 years at the four of the five patients had panuveitis. the fifth one had intermediate uveitis. the two children younger than 5 years showed the clinical triad of eye, joint and skin disease. Uveitic complications included corneal band keratopathy, cataract, posterior synechiae, macular scars and choroidal neovascularisation. Vision-limiting complications were found in six of nine eyes at the first visit. Conclusion: In childhood sarcoidosis two clinical courses can be differentiated. Younger children aged up to five years express a clinical triad of arthritis, skin lesions In the older group of patients, the clinical course is more similar to that of adults. Rapid diagnosis and quiescence of disease are of particular importance to improve the long-term visual prognosis.