Sarcoidosis is a multisystem granulomatous disease of unknown aetiology, most commonly affecting young adults. Divergent prevalence rates and clinical appearances in different races indicate the existence of predisposing genes, with probably a major effect of the major histocompatibility complex. The diagnosis is relatively rare in children. Lesions can occur in almost any tissue or organ but the lungs, lymph nodes, eyes, skin and liver are the most commonly involved. Clinical symptoms are non-specific and often minor. The diagnosis can only be supported by typical histopathological findings with non-caseating epithelioid-cell granulomas. The prognosis seems to be more severe in younger children and in cases of multi-organ involvement. Corticosteroids are the therapeutic agents most commonly used and are indicated in cases of significant lung or eye lesions, cardiac, neurological, or multiorgan involvement. Close monitoring is mandatory during and after treatment because relapses are common.