Purpose of Review <p>To provide an update on the literature regarding diagnosis and management of immune checkpoint inhibitor myocarditis.</p> Recent Findings <p>The diagnosis of immune checkpoint inhibitor myocarditis has evolved to include more reliance on performing endomyocardial biopsy to clarify the diagnosis in selected cases. Additionally, there is recognition of a spectrum of disease both clinically and on endomyocardial biopsy suggesting that there is a range of severity from mild to fulminant. The treatment of immune checkpoint inhibitor myocarditis is shifting towards increased use of additional immunosuppressive medications as steroid sparing agents. There are increased studies including two randomized controlled trials evaluating abatacept in the treatment of immune checkpoint inhibitor myocarditis.</p> Summary <p>This review summarizes the latest literature regarding diagnosis and management of immune checkpoint inhibitor myocarditis and provides our experience and approach to this rare but potentially fatal condition.</p>