Sarcoidosis is a multisystem granulomatous disease of unknown etiology and with variable presentation. Skin, lymph nodes, lungs, eyes and the central nervous system are mostly involved. Cardiac sarcoidosis (CS) is a rare condition with clinical manifestations in about 5% of patients. Since it increases the risk of acute cardiac failure, ventricular arrhythmia, conduction disturbances and even sudden death, it aggravates markedly the prognosis. The early diagnosis of CS is difficult, requiring the use of diagnostic tools such as electrocardiographic monitoring, two-dimensional echocardiography, radionuclide scan, cardiac magnetic resonance imaging, positron emission tomography and endomyocardial biopsy. Once the diagnosis of CS is established, there is a need for early corticosteroids treatment, with or without immunosuppressive therapy, to prevent deterioration of cardiac function. In patients with refractory ventricular tachyarrhythmia, markedly reduced left ventricular ejection fraction and high risk of sudden death, prophylactic insertion of a pacemaker or implantable defibrillator is recommended. We had the opportunity to treat a patient with CS and to review the currently accepted diagnostic and treatment approach.