Introduction: Low-flow vascular malformations are congenital abnormalities of the veins, capillaries or lymphatic vessels or a combination of the previous. Compressive garments are frequently used as a first-line treatment option for low-flow vascular malformations of the extremities with the purpose of relieving symptoms. Yet, the benefits and harms of compression stockings remain unclear. Methods: A systematic search was performed in MEDLINE, Embase, Cochrane Central Register of Controlled Trials including a hand search for studies measuring the benefits and harms of compression garments in treating low-flow vascular malformations. Two investigators performed study selection, quality assessment and data extraction independently. Results: Of the 565 studies found, eventually five (totalling 101 patients with venous malformations or Klippel–Trenaunay syndrome receiving compression therapy) observational studies were included in the systematic review. Although the overall quality of these studies was poor, results suggest that compression garments might lessen intravascular coagulation, improve symptoms and appearance, diminish oedema, and protect against minor trauma. None of the studies quantified any harms of compression therapy. Conclusion: Even though compression therapy is commonly used in the treatment of low-flow vascular malformations, available literature does not provide high-quality evidence to validate its use. We therefore advocate the need for prospective comparative trials with standardised outcome measures to study the benefits and harms of this treatment option.