Background: Venous malformations (VM) are rare vascular malformations. They grow progressively with the patient and don’t regress spontaneously. Several high specialized centres, which often treat these, recommend multidisciplinary approaches, but there is no recognized consensus on the treatment of these lesions. Goal: Shed light on VM with emphasis on the head and neck region and what treatment is being given. Method: A systematic literature search was conducted. To get a synopsis of available information a search was conducted in UpToDate using the term: “Vascular anomalies” and “Venous malformations”. The main extensive search was conducted in PubMed using the terms: “Venous malformations”, “Head and neck” and “Vascular surgical procedures”. Based on title and abstract relevant articles reviewing VM were included. Articles reviewing vascular malformations were also included, those reviewing extracranial were excluded. The search was saved and updated 28.01.2020 to ensure that the latest articles were included. Results: No randomized clinical trials were found. Most articles are based on retrospective studies. Standard treatment for VM is primarily sclerotherapy, often in combination with surgery, surgery alone, laser treatment, compression garment and/or conservative observation. Some studies have suggested algorithms for the management of these lesions, but there is still no recognized consensus. Conclusion: There is a need for randomized clinical trials assessing the different treatment modalities. So far, drug treatment has proven to be safe and effective for a small patient population; possibly this will be the future standard treatment.