Cardiac amyloidosis, an uncommon disease, has been reported to manifest as congestive heart failure (CHF) and/or various arrhythmias. Herein, we report a case of CHF and sick sinus syndrome. The patient, a 66-year-old man, was admitted to the National Taiwan University Hospital because of dizzy spells and recurrent syncope. Electrocardiogram showed a sinoatrial block, first degree atrioventricular block, right bundle branch block and low-voltage Q wave, R wave and S wave (QRS) complex. Prolonged corrected sinus node recovery time was documented by an atrial pacing study. A permanent pacemaker was implanted for the patient's bradyarrhythmia, but he developed progressive heart failure. Echocardiography revealed a normal-sized ventricular chamber, concentric left ventricular hypertrophy with a "granular sparkling" appearance of the myocardium, and impaired diastolic and systolic function of the left ventricle. Despite aggressive treatment, the patient expired due to intractable heart failure. Postmortem needle aspiration revealed amyloidosis involving the heart, lung and skin. We conclude that cardiac amyloidosis should be considered in elderly patients with conduction disturbance and unexplained congestive heart failure.