PEComas represent a rare class of mesenchymal tumors, with different primary locations. There are less than 100 cases of uterine PEComas published in English literature until now and information considering imaging features of these PEComas is very limited, focusing on CT and MRI and not as much on ultrasounds (US). The authors present here a case of rapidly growing uterine PEComa, with local invasive potential and recurrence, and the review of literature on US characteristics of PEComas. Harboring a hyperechogeneous heterogeneous aspect with no clear separation from the adjacent uterus on the whole boundary, with an extremely rich central vascular network, with low impedance and a rapidly growing profile, this tumor does not show the classic US appearance of malignant PEComas, which are generally easily confused with leiomyomas. However, even if this pattern did not allow the authors to anticipate the histopathological result, it offered clear clues about its invasiveness potential.