We report the first case of multiple plexiform tumourlets showing focal vascular invasion suggesting that, unlike what the common and accepted opinion would advocate, these tumours may have a more aggressive behaviour. However, the prognosis of this variant of epithelioid leiomyoma remains unknown, due to its rarity. The main differential diagnosis of this entity are discussed, including endometrial stromal sarcoma exhibiting epitheloid cytomorphology, intravenous leiomyomatosis of epitheloid variant.