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Resumen: Se presenta el caso de inusual crecimiento intracardíaco de leiomiomatosis intravascular gigante en una mujer de 38 años, en quien durante evaluación prequirúrgica de cirugía abdominal se documentó de manera incidental una masa en cavidades cardiacas derechas. Dada su presentación infrecuente y las dificultades en el abordaje diagnóstico y terapéutico, se realiza posterior revisión de la literatura. Abstract: The case is presented of an unusual intracardiac growth of a giant intravascular leiomyomatosis in a 38 year-old woman. During a pre-surgical evaluation for abdominal surgery, the incidental finding of a mass in the right cardiac cavities was noted. Given its uncommon presentation and the difficulties in the diagnostic and therapeutic approach, a subsequent review of the literature was performed. Palabras clave: Ecocardiografía, Tumores, Diagnóstico, Keywords: Echocardiograph, Tumours, Diagnosis