Juvenile systemic sclerosis (JSSc) is a rare but severe multi-system connective tissue disease of unknown etiology. It is one of the most difficult to treat rheumatic diseases in childhood and characterized by thickening and fibrosis of the skin and associated with fibrosis of internal organs. Eye involvement has rarely been reported. In a 7-year-old African girl, who presented with chorioretinitis and subsequently developed JSSc we discuss the possible association of chorioretinitis with JSSc and the putative implications of scleroderma vascular disease in the development of this complication and review the existing literature.