The pathological term malformation is based on the clinical and histological appearance of the vascular channels. These channels are histologically representative of arteries, capillaries, veins, lymphatics, or a combination of these structures. Molecular studies suggest that vascular anomalies are caused by dysfunctional signaling processes that regulate proliferation, apoptosis, differentiation, maturation, and adhesion of vascular cells. 4 Malformations are structural anomalies based on inborn errors of vascular morphogenesis. These lesions are noted to have high endothelial cell turnover. Malformations are further categorized into low-flow and high-flow lesions. This review will focus on the presentation and treatment of low-flow vascular malformations. In general, the majority of vascular malformations are sporadic. Conversely, heritable forms can be found in Rendu-Osler-Weber syndrome, Fabry's disease, and ataxia-telangiectasia.