Atrial myxomas are the most common benign primary tumor of the heart and occur in as many as 3 in 1000 patients. These tumors are a major cause of patient morbidity and mortality. Although the majority of atrial myxomas occur in the left atrium, 3 separate familial myxoma syndromes can result in multiple myxomas in atypical locations. Approximately 50% of patients with myxomas may experience symptoms due to central or peripheral embolism or intracardiac obstruction, but 10% of patients may be completely asymptomatic. Screening for myxomas should involve a thorough history and physical examination and a transthoracic and/or transesophageal echocardiogram. Transthoracic echocardiography is approximately 95% sensitive for the detection of cardiac myxomas, and transesophageal echocardiography approaches 100% sensitivity. Though the majority of atrial myxomas are sporadic, it is imperative that first-degree relatives of patients with documented myxomas undergo screening for occult myxomas. Surgical removal of the myxoma is the treatment of choice and usually curative; however, myxoma recurrence does occur and is most frequently associated with a familial syndrome.