Primary cardiac sarcoma is an uncommon entity and is related with poor long-term survival. Presented here is an unusual case of right atrial leiomyosarcoma which almost completely occluded both the right atrium and ventricle and destroyed the tricuspid valve. Multimodality imaging was used to characterize the mass and guide surgical management. A combination of two radical surgical excisions and both radiation and chemotherapy resulted in a better long-term outcome. The patient was one of the longest survivors.
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