Chondrosarcoma is a rare entity of malignant tumor which arises from cartilaginous tissue, and the literatures on this disease are scarce. The first-line of treatment for cardiac chondrosarcoma is surgery. Due to early local recurrence and distant metastasis, the prognosis is poor even after complete surgical excision. We present a case of chondrosarcoma in the left atrium causing functional mitral stenosis which required urgent surgical intervention, and the successful treatment outcome.