Inflammatory cardiomyopathy (ICMP) is characterized by myocarditis associated with cardiac dysfunction. Clinical presentation may include acute heart failure and cardiogenic shock, chronic heart failure, ventricular tachyarrhythmias or may mimic an acute coronary syndrome. Inspite of the recent improvements in non-invasive diagnostic techniques, such as cardiac magnetic resonance, the diagnostic gold standard is still the endomyocardial biopsy. In the last years, classical histological Dallas criteria have been significantly improved by the introduction of immunohistochemical and molecular biology techniques. Recent findings using these new diagnostic tests resulted in increased interest in ICMP and a better understanding of its pathophysiology, the recognition in overlap of virus-mediated damage, inflammation, and autoimmune dysregulation. Moreover, there is growing evidence that the treatment of these patients with specific strategies may be effective only if based on their immunological and virological characterization. Several studies suggest that patients with autoimmune ICMP can benefit from immunosuppressive treatment and immunoadsorption, as well as high dose immunoglobulins, showing a significant improvement of left ventricular function and heart failure symptoms. On the other hand, immunosuppressive treatment can be deleterious for ICMP patients with evidence of viral persistence, while antiviral treatments have been proven effective in this subgroup. The present review summarizes the recent advances in the diagnosis and risk stratification of ICMP, and reviews patents and treatment options for these patients.