A rare case of primary cardiac leiomyosarcoma was diagnosed in a 21-year-old man who presented with a groin mass thought to be a sebaceous cyst. Histopathology revealed a high-grade pleomorphic leiomyosarcoma. Combined positron-emission and computed tomography showed a large metabolically active left atrial mass with multiple metastases. Major debulking resection was undertaken, followed by radiation and chemotherapy. At 13 months postoperatively, limited spread has been detected, and the patient had no limitation in daily life.
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