Vascular malformation is a group of developmental malformations originating from blood vessels and/or lymphatics that can invade multiple organs and systems, causing pain, disfigurement, dysfunction, and even death. At present, the treatment methods of vascular malformation are various, but the effect is not satisfactory. Sirolimus, a mammalian target protein receptor inhibitor of rapamycin, has been shown to be effective in the treatment of vascular malformations. In this paper, the mechanism, application, effect and possible side effects of sirolimus in treating vascular malformation were reviewed, in order to provide a new idea for treating such diseases.