The often inexorable growth and expansion of congenital vascular malformations can result in substantial morbidity and, in some cases, premature death of these patients. Despite this, patients suffering from such lesions are often erroneously diagnosed and/or inadequately treated, due to a lack of expertise among primary care practitioners as well as specialists. Venous malformations are the most common type of congenital vascular malformations. Over the last two decades management of these lesions has significantly improved, predominantly due to the introduction and implementation of multidisciplinary team concept as well as improvement in diagnostic and treatment modalities. Relatively recently genetic studies are providing more insights into underlying pathophysiological mechanisms responsible for the development and progression of venous malformations and pharmacotherapy is becoming extensively evaluated for safety and efficacy in the treatment of these often challenging vascular lesions.