Cardiac valvular tumors are very infrequent lesions constituting about 10% of the primary cardiac neoplasms. Among the valvular tumors, lipomatous hamartoma represents a very rare and distinct pathological entity. Retrieval of the literature reveals only eight reported cases, and there is lack of information regarding their clinical features. Herein, we describe a patient with a mass on the tricuspid valve detected incidentally and diagnosed to be a "lipomatous hamartoma." To obtain a better understanding of the nature of these unusual lesions, we reviewed the literature and presented their clinicoanatomical characteristics together with diagnostic and therapeutic approaches.
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