Skeletal muscle inflammation is the feature shared by all forms of myositis. However, the muscle damage ranges in severity from asymptomatic to responsible for severe weakness. In addition, myositis usually occurs as a systemic disease that affects multiple organs. More specifically myositis should be considered in patients with muscular, cutaneous, pulmonary, and articular manifestations. The definitive diagnosis and classification of myositis has benefited considerably in recent years from the identification of characteristic autoantibodies. Nonetheless, a muscle biopsy is often necessary to confirm the diagnosis, and the differential diagnoses may raise challenges that require evaluation at a myositis referral center. The management depends on the type of myositis. Treatments should be provided for both the systemic complications (involving the lungs, heart, gastrointestinal tract, and/or joints) and the comorbidities (cancer and risks factors for cardiovascular disease, thromboembolism, and fractures), which together determine the prognosis. Many drugs are available for treating myositis. Findings from randomized controlled trials will help to use them optimally.