Abstract Background Cardiac hemangiomas are rare cardiac neoplasia usually diagnosed in autopsies, from being asymptomatic to debuting with sudden death. The largest hemangioma published in the literature is of 130 mm size, we present the following case of a successful cardiac hemangioma excision of 280 × 35 mm in size, diagnosed due to recurrent cardiac symptoms. Case presentation A 48-year-old female patient, Jehovah’s Witness, with no previous diagnoses, is admitted due to recurrent syncopal episodes in the previous months. A transthoracic echocardiogram diagnosed a tumor in the right atrium and inferior vena cava producing a diastolic right ventricular, with preservation of the left ventricular ejection fraction at 55%. Given the high mortality risk, a surgical intervention was performed immediately. Successful excision was completed confirming a 280 × 35 mm mass without any complications, consistent with hemangioma on histopathology. Postoperative recovery showed no recurrence or complications. Conclusions Cardiac hemangiomas are rare and its clinical course can be varied from patient to patient. We present excision of a large cardiac mass with a high mortality risk due to its size and the patient’s spiritual beliefs.