Background <p>Vascular malformations comprise a&#xa0;broad spectrum of vascular anomalies that can affect any part of the vascular system. Consequently, the range of possible symptoms, complaints and complications is very diverse. In addition to established forms of pharmacotherapy, such as pain management and anticoagulation, understanding specific genetic alterations in the PI3K/AKT/mTOR and RAS signaling pathways has opened up new targeted treatment strategies.</p> Objective <p>Review of current pharmacological treatment options for symptomatic vascular malformations with special focus on novel targeted treatment approaches.</p> Methods <p>Analysis of the current literature and clinical experience regarding conventional and experimental pharmacological treatment approaches.</p> Results <p>Besides the treatment of complications, such as coagulation disorders and pain, targeted therapies have become established in recent years. The mTOR inhibitor sirolimus shows good efficacy in venous and lymphatic malformations. For PIK3CA-mutated malformations, alpelisib is being investigated in clinical trials. For arteriovenous malformations MEK inhibitors, such as trametinib are being used in individual treatment approaches.</p> Conclusion <p>Treatment decisions should be made in an interdisciplinary setting, taking the type of malformation, symptoms, location and molecular pathogenesis into account. Targeted therapies should preferably be conducted within clinical trials. Further studies will be necessary to establish evidence-based forms of pharmacotherapy.</p>