Dermatomyositis (DM) is an idiopathic chronic, inflammatory myopathy with characteristic cutaneous manifestations in addition to symmetrical proximal muscle weakness and systemic involvement. In addition to the classic presentation, variants such as amyopathic and hypomyopathic DM are also recognized. The pathogenesis is believed to be multifactorial with immune-mediated vasculopathy, genetic predisposition, and environmental triggers playing a crucial part. Cutaneous signs include heliotrope rash and Gottron’s papules, confluent macular violaceous erythema, and rare findings such as mechanic’s hands, ulcerations, and poikiloderma. Systemic involvement includes respiratory, cardiac, gastrointestinal, and musculoskeletal systems. Juvenile DM shows distinct clinical features including a higher incidence of calcinosis and lower malignancy risk. Diagnosis relies heavily on clinical acumen, and early recognition and intervention are essential to reduce morbidity and improve outcomes.