Scleroderma is a complex, immune-mediated autoimmune disorder characterized by heterogenous organ involvement, progressive fibrosis, and vascular abnormalities. Genetic factors, environmental triggers, and abnormal immune responses contribute to the development of the disease. Its pathogenesis is complex and involves initial endothelial damage, an inflammatory infiltrate and fibrosis. Dysregulated endothelial cell function and vascular damage further contribute to the pathophysiology, causing tissue ischemia and organ damage. Clinical features are distinct, affecting the skin, gastrointestinal tract, lungs, kidneys, heart, and musculoskeletal system. Skin fibrosis is the hallmark feature. Other features include Raynaud’s phenomenon, digital ulcers, gastroesophageal reflux disease, pulmonary artery hypertension, interstitial lung disease, and arthralgia. Unfortunately, a curative treatment is still not available for the disease. Management of organ complications remains a wise approach. Treatment goals include preventing complications and preserving organ function. Medications such as immunosuppressants, vasodilators, calcium channel blockers, endothelin receptor antagonists, and antifibrotic agents may be used to target underlying pathogenesis.