The presentation of scalp metastases from leiomyosarcoma of the vena cava is an extremely infrequent event. There are no other publications that describe such finding and very few of leiomyosarcoma in vessels. About this event we have reviewed the English literature describing studies on scalp metastases and skin metastases in general: their incidence, origin, clinical appearance, meaning, and diagnosis. The case we describe would be the second one presented worldwide because, as far as we know, it has been only one more published in 2005.