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Abstract Background Cardiac Hibernomas are very rare benign tumors and usually remain asymptomatic. Neonatal cardiogenic shock due to cardiac tumors is extremely very rare. Until this date a few cases of cardiac hibernoma have been reported in the literature. Transthoracic echocardiography help in the differential diagnosis, but the definitive diagnosis is histological. The management strategy is not clearly codified. The Aim is to report and discuss the clinical features of a cardiac Hibernoma and review the relevant literature. Case presentation We describe a case of a 2-day-old Caucasian full-term male neonate admitted in neonate intensive care with cardiogenic shock, having fluid resuscitation and inotropic drugs. Ventilatory support was started immediately with the subsequent reestablishment of normal blood pressure. Then he was transferred to the echocardiography laboratory. Transthoracic echocardiography showed two echogenic masses in the right atrium and right ventricle. The masses were extended to the pulmonary trunk. Pulmonary artery flow measurements showed the presence of pulmonary and tricuspid obstruction. Surgery was rapidly considered since the baby was hemodynamically unstable. Intraoperative evaluation showed a mass embedded in the interventricular septum that occupy the right ventricular cavity and the right atrium. The tumor involved also the chordae of the tricuspid. Partial resection was done. Tricuspid valve repair was performed by construction of new chordae from the autologous pericardium. The specimen was sent for histopathological analysis. The baby died immediately after surgery. Histological examination of the surgical specimen revealed clear multivacuolated cells filled with lipid droplets and granular intense eosinophilic cytoplasm which confirms the diagnosis of Hibernoma. Conclusion Cardiac Hibernomas are rare benign tumors. The prognosis and treatment strategy is closely dependent on the location, initial clinical presentation and possible complications. The prognosis can be unfavorable if the tumor was obstructive and infiltrate the myocardium.