Glomus tumor is a soft tissue neoplasm usually observed as a solitary, or sometimes multicentric painful mass, that rarely occurs extracutaneously. We describe a rare case of malignant glomus tumor of the heart in a 64-year-old male diagnosed with a left ventricular mass. Echocardiography and color flow Doppler revealed a large echogenic mobile structure in the left ventricular cavity that was surgically resected. The histopathologic diagnosis was malignant cardiac glomus tumor. We describe the histopathology, differential diagnosis and clinical presentation of this extremely rare primary cardiac tumor.

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