In inflammatory dilated cardiomyopathy and myocarditis, there is apart from heart failure and antiarrhythmic therapies no alternative to an etiologically driven specific treatment. Their prerequisites are noninvasive and invasive biomarkers including endomyocardial biopsy and PCR on cardiotropic agents. This review deals with the different etiologies of myocarditis and inflammatory cardiomyopathy including the genetic background, the predisposition for heart failure and inflammation. It analyses the epidemiologic shift in pathogenetic agents in the last 20 years, the role of innate and acquired immunity including the T cell and B cell driven immune responses. On this basis, it summarizes phases and clinical faces of myocarditis. It gives an up-to-date information on current treatment options starting with heart failure and antiarrhythmic therapy. Although inflammation can resolve spontaneously, often specific treatment directed to the causative etiology is required. For fulminant, acute and chronic autoreactive myocarditis immunosuppressive treatment is beneficial; for viral cardiomyopathy and myocarditis, IVIG can resolve inflammation and is as successful as interferon therapy in enteroviral and adenoviral myocarditis. Eradication of parvovirus B19 and HHV6 myocarditis is still a problem by anyone of these treatment options. The potential of stem cell therapy has to be tested in future trials. In perimyocardial disease, a locoregional approach with high local doses and low systemic side effects have been shown highly efficient by intrapericardial treatment of triamcinolonacetate facilitated by pericardioscopy for adequate etiopathogenetic diagnosis.