Sarcoidosis is rare multi-system granulomatous inflammatory disorder. It is reported to present by two distinct presentations in childhood. Sarcoidosis presents in the second decade of life by pulmonary infiltration, lymphadenopathy and hepatosplenomegaly, and rarely before the age of 4 years by typical triad of uveitis, rash and arthritis. We report a child 1 year and 9 months with sarcoidosis who presented by one month fever, hepatosplenomegaly, uveitis, pulmonary infiltration and elevated levels of angiotensin-converting enzyme. Chest computerized tomography revealed multiple enlarged mediastinal and multiple hilar lymphadenopathy. A right inguinal lymph node biopsy showed non-caseating granuloma. Other etiologies were investigated for and ruled out. Hence, sarcoidosis can present in children before 4 years with uveitis, hepatosplenomegaly, pulmonary infiltrates, mediastinal and hilar lymphadenopathy, in the absence of rash and arthritis. Sarcoidosis in children is rare and clinical spectrum can vary considerably. Diagnosis of sarcoidosis should be suspected in any child with uveitis.