Abstract A review of the symptomatology of right atrial myxoma is given, based on 33 cases from the literature and on two cases studied by us.This diagnosis should be considered in any patient with isolated right heart failure without pulmonary congestion, pulmonary disease, or pulmonary stenosis.A tricuspid murmur, intermittent cardiac or cerebral symptoms or radiographic demonstration of non‐valvular intracardiac calcifications should increase the suspicion.Selective right atrial angiocardiography is the diagnostic method of choice. The diagnosis cannot be excluded without this examination, which always should be performed in any patient with isolated tricuspid valvular disease.