Cardiac sarcoidosis (CS) is a rare and under-recognized clinical entity that requires a high level of suspicion and low threshold for screening in order to make the diagnosis. CS may manifest in a variety of ways, and its initial presentation can range from asymptomatic electrocardiographic abnormalities to overt heart failure to sudden cardiac death. The aim of this literature review is to provide a comprehensive overview of CS, with an emphasis on clinical manifestations and special diagnostic and management considerations, while highlighting recent studies that have provided new insights into this unique disease.