Systemic sclerosis is a diffuse disorder of connective tissue in which there are inflammatory, fibrotic and degenerative changes in the skin and many internal organs. We describe a case of systemic sclerosis type "acrosclerosis" in a six years old child with Raynaud's phenomenon and gastrointestinal involvement. In the course of illness he presented severe digital ulcerations in the hands and in the feet, responsive to a calcium channel blocking agent nifedipine treatment.