Background Takotsubo syndrome (TTS) often develops following various types of stressors including cardiac surgery, and may be fatal in rare cases. Case summary We report a rare case of right atrial papillary fibroelastoma that was complicated by fatal takotsubo syndrome after successful resection. The patient was evaluated for chest pain and right atrial mass that was confirmed by computed tomography and trans-esophageal echocardiography. Pathology findings were consistent with papillary fibroelastoma of the tricuspid valve. Three weeks after successful surgery, the patient was admitted due to cardiogenic shock with echocardiographic findings of apical ballooning and left ventricular outflow tract obstruction, consistent with takotsubo syndrome. Patient died one hour after her admission despite optimal medical therapy.