Primary tumours of the heart are reported with a prevalence rate of around 0.01% in collective autopsy series. Malignant tumours account only for 25% of all primary cardiac neoplasms most of which are sarcomas and the most common cardiac sarcoma is Angiosarcoma.
We present a case report of a 46 year old female, who had history of dyspnoea, chest pain, weight loss, fever and body ache. Cardiac MRI revealed a huge Angiosarcoma of Right Atrium (10.8 x 8.2 x 6.6 cm) with extension into the Right Ventricle. Diagnosis of Angiosarcoma was established on histology. The literature on Angiosarcoma of heart is reviewed.