Giant cell myocarditis is a rare and fatal heart disease in previously healthy young patients. We report the case of a 43-year-old patient presenting unstable acute congestive heart failure as a consequence of myocarditis who was supported for four days by an extracorporeal membrane oxygenation. While no cardiac recovery was observed and no viral and autoimmune causes of myocarditis were found, he underwent successful orthotopic heart transplantation in emergency. Giant cell myocarditis was diagnosed on the explanted heart. The patient has been on a triple-immunosuppression therapy with no signs of recurrence of the disease or rejection 16 months after surgery. This experience is compared with published cases and implication of diagnosis and treatment are discussed.