| Summary: | The group of congenital limb overgrowth syndromes associated with vascular malformations include various disorders, often with variable clinical expression. Klippel-Trenaunay syndrome is the most common syndrome in this group, compared to other similar disorders such as Proteus, Parkes-Weber, Sturge-Weber, Cobb, Maffucii and CLOVES syndromes. We present a case of a 55-year-old male who presented with swelling, pain, heaviness, numbness and increased local temperature, involving the right upper extremity and the right side of the chest wall and associated with a livid erythema. The patient had several comorbidities including arterial hypertension and ischemic heart disease. He also had a family history of diabetes mellitus. A multidisciplinary team-based treatment approach, including physicians with expertise in various medical and surgical specialties was applied with the patient being “the unifying focus” of all these experts and their efforts. A personal approach tailored to the patient’s preferences was the key to a successful treatment.
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