LMs can present either as an isolated vascular anomaly or as part of overgrowth syndromes that arise due to mutations in the PI3K/AKT pathway, such as Proteus syndrome, Klippel-Trenaunay syndrome (KTS) and congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies (CLOVES) syndrome [4]. The gene discussed is PIK3CA; the disease is angioosteohypertrophic syndrome.