There are 2 distinct forms of sarcoidosis in childhood. We describe the clinical picture of 6 patients with sarcoidosis of the early onset type. All patients had their disease onset during the first 4 years of life. In contrast to the black predominance reported in adult patients, our series comprised 5 whites and one Latin-American. The triad of rash, arthritis, and uveitis seems to be characteristic for this form. The symptoms in our patients were comparable to those in the literature. However, a long followup (9-23 yrs) allowed us to recognize severe complications of the disease, previously not well known, such as blindness (4 patients), growth retardation (3 patients), heart involvement (2 patients), renal failure (1 patient), and even death in one patient. Pulmonary involvement was present only in one patient late in the disease course. This condition may be overlooked, and is sometimes misdiagnosed, due to its rarity and to its similarities with juvenile arthritis. However, the clinical characteristics of the 2 diseases are different. Early and correct diagnosis is important in light of the severe disease prognosis, and the possible therapeutic options.