Resumen Los sarcomas originados en las paredes de los grandes vasos son tumores de escasa presentacion. El leiomiosarcoma de la vena cava es una enfermedad compleja que requiere ser manejado por un grupo multidisciplinario. Su tratamiento es quirurgico. Poco se conoce de sus resultados oncologicos. Se presenta el caso de una paciente de 56 anos, con diagnostico de leiomiosarcoma de la vena cava inferior, su evaluacion, manejo quirurgico y seguimiento a corto plazo. Se hace una revision de la literatura existente hasta la fecha, evaluando ademas la fi siopatologia y la necesidad de reconstruir la vena cava inferior.© 2013 Instituto Nacional de Cancerologia. Publicado por Elsevier Espana, S.L. Todos los derechos reservados. Leiomyosarcoma of the inferior vena cava: a case report and review of the literature Abstract Sarcomas Sarcomas originating in the walls of the large vessels are tumors of unusual presentation. Leiomyosarcoma of the vena cava is a complex disease that needs to be managed by a multidisciplinary team. Its management is surgical. Little is known of their oncologic results. We report the case of a 56 year-old patient, diagnosed with leiomyosarcoma of the inferior vena cava, including her evaluation, surgical management, and short-term follow-up. An updated review of the literature is presented, along with an evaluation of the pathophysiology and the need for reconstruction of the inferior vena cava.© 2013 Instituto Nacional de Cancerologia. Published by Elsevier Espana, S.L. All rights reserved.