Systemic sclerosis (SSc) is a complex autoimmune connective tissue disorder characterized by fibrosis, vascular abnormalities and immune dysregulation. It affects multiple organ systems and presents with a wide spectrum of clinical manifestations. Cutaneous involvement progresses through ill-defined stages, with Raynaud’s phenomenon often being the earliest sign. Facial changes, digital ulcers, sclerodactyly, pigmentary alterations, nail abnormalities and calcinosis are key dermatologic features. Systemic manifestations include gastrointestinal involvement, pulmonary complications such as interstitial lung disease and pulmonary hypertension, cardiac abnormalities, renal crisis and musculoskeletal features. Given its high morbidity and mortality, early diagnosis is critical, and dermatologists play a pivotal role in identifying initial cutaneous signs. Advancements in understanding the pathogenesis and risk factors have improved management strategies, but SSc remains a challenging condition with significant clinical variability.