Histologically benign soft-tissue chondromas have been reported at many anatomical sites but are an uncommon cause of soft tissue mass lesions in childhood, accounting for less than 1% of cases. The most frequent sites for extraosseous soft-tissue chondromas are the hands and feet. For the extremely rare visceral chondromas, the site can be lung, where they may represent a component of Carney's syndrome of extra-adrenal paraganglioma, pulmonary chondroma, and epithelioid leiomyosarcoma of the gastrointestinal tract. Primary cardiac chondromas are exceptionally rare in patients of any age although cardiac chondrosarcoma, both primary and metastatic, is well reported. We present a case of a teenage boy with a fatal cardiac chondroma