Twenty-five percent of primary cardiac tumors are malignant, and 95% of these are due to sarcoma [
3]. Angiosarcoma is the most common type and accounts for around 40% of sarcomas, others include undifferentiated sarcoma (24%), malignant fibrous histiocytoma (14–24%), leiomyosarcoma (8%), osteosarcoma (6%), and least of all, spindle cell sarcoma [
1,
3,
4]. Cardiac sarcoma can present with sudden onset, intermittent or positional symptoms of chest discomfort, dyspnea, orthopnea, cough, or syncope, heart failure, pericardial effusion and tamponade, arrhythmias, valvular abnormalities, or obstructive symptoms depending on heart chamber involved. Diagnosis is usually established using non-invasive imaging modalities such as transthoracic or transesophageal echocardiography. CT and MRI are useful for complete assessment of tumors occupying the entire cardiac chamber, tumors infiltrating myocardium or pericardium, or tumors with adjacent great vessel involvement [
5]. At time of diagnosis, up to 80% of spindle cell sarcomas exhibit evidence of metastasis [
2]. Tumor emboli are common and cause distant metastases involving bone, peritoneum, liver, and mesenteric lymph nodes [
2]. Furthermore, spindle cell sarcoma is a highly aggressive and can rapidly metastasize. In conclusion, we report a rare case of spindle cell sarcoma obstructing the right ventricular outflow tract. Although treatment of this neoplasm of heart remains challenging due to its rarity and aggressive nature, radical surgical excision remains the mainstay treatment.