BACKGROUND Myxomas are rare benign tumors of the heart. These tumors are commonly located in the left atrium, but rarely can affect the right side of the heart. Although it is a relatively rare tumor, it is the most common primary cardiac tumor, accounting for 75-80% of them. Secondary or metastatic cardiac tumors are much more common than primary tumors, accounting for more than 95% of cardiac tumors. CASE REPORT A 38-year-old woman presented with shortness of breath and syncope. Upon investigation, she was found to have a right atrioventricular myxoma. It was associated with tricuspid regurgitation, right-sided heart failure, and pulmonary hypertension. The syncopal attacks and shortness of breath resolved completely after tumor resection. Tricuspid regurgitation (grade 1) and mild pulmonary hypertension (right ventricular systolic pressure 35 mmHg) remained as sequelae of delayed presentation. These may be due to recurrent embolization of tumor fragments to segments of the pulmonary artery. CONCLUSIONS Cardiac myxomas should be considered in patients presenting with unexplained shortness of breath and syncope. Early diagnosis and tumor resection may prevent serious complications such as pulmonary hypertension and right-sided heart failure.