Abstract: Scleroderma may occur in childhood with different characteristics. The present study shows the picture of scleroderma in Iranian children and compares the characteristics in children and adults. Methods: 56 scleroderma patients under 16 were compared with 217 adult scleroderma seen since 1975. Data were compared with Chi square test. Results: Girls were 44 (79%) and boys 12. The initial presentation was: Raynaud's phenomenon 68%, skin edema 27%, limb skin sclerosis 66%, facial skin sclerosis 23%, GI involvement 18%, joints 32%, and respiratory symptoms 7%. The overall symptoms were as follow: Constitutional symptoms 37%, joint manifestations in 64%, skin manifestations 96%, hypo-pigmentation 14%, pitting ulcers 32%. X-rays showed: Acro-osteolysis 18%, pulmonary involvement 12%, esophageal 11%, anemia 27%, raised ESR in 45%, systemic sclerosis 78%, limited 14%, morphea 7%, and linear 7%. Conclusion: Skin as the initial manifestation was more frequent (p<0.01) while pitting ulcer was less frequent in children (p< 0.01). Esophageal and lung problems were more frequent (p<0.01), while osteolysis was less frequent (p=0.06) in children than in adults.