Systemic sclerosis is a connective tissue disease involving primarily the skin and subcutaneous tissue but may involve other organ systems such as the lungs, gastrointestinal tract, and heart. Systemic sclerosis is a rare childhood disease. The age of onset for patients with systemic sclerosis has its peak between 30 and 50 years of age. Only 1 to 2% of all patients with systemic sclerosis have disease onset before the age of 10 years of age. We report a case of systemic sclerosis in a 7- year-old female who showed Raynaud‘s phenomenon as an initial manifestation. (Korean J Pediatr 2004;47:214-216)