Abstract: Objective: Systemic sclerosis is a generalized disorder of connective tissue disease. This study surveyed the clinical manifestations of systemic sclerosis in early (<3 years) and late (>6 years) phases of limited and diffuse variants. Methods: Nineteen patients with diffuse and thirty-four cases with limited form of disease were selected consecutively from 1992 to 1998. Sixty-five clinical data were evaluated and then analyzed by MC-Nemar chi square test between two forms in both phases. Results: (1) There were no significant differences of clinical manifestations in both phases of diffuse disease. (2) Telangiectasias (p < 0.0005), Raynaud's phenomenon (p < 0.007). hyperpigmentation (p < 0.01), and esophagitis (p < 0.02) were obtained significantly more in late phase of limited form. (3) Comparison between two forms in early phase showed there were no significant differences in clinical presentations. (4) Motor dysphagia was only manifestation that significantly seen (p < 0.02) more in diffuse form of the late phase. Conclusion: Comparison between our data and Pittsburg university study shows that digital ulcers were seen more in our patients in two phases of both forms (p < 0.05), Calcinosis and telangiectasias were seen more in Pittsburg in limited form in both phases (p < 0.05).