On the basis of a personal observation and from a complete review of the existing literature, clinical and instrumental findings of right atrial myxoma are described. The clinical presentation appears to be rather characteristic. The presence of systemic symptoms, peripheral venous hypertension and/or right heart failure without apparent cause are a clue to the diagnosis especially in an adult woman. The presence of abnormal sounds and/or murmurs in the tricuspid area and intermittence of the signs of obstruction are further support to the diagnosis. The diagnosis can always be made non-invasively by echocardiography, though some pitfalls seem to be inherent to the M-mode technique. The accuracy of two-dimensional echocardiography in the diagnosis of the right atrial myxoma is such that no further investigations are necessary before surgery.