Introduction The phakomatoses are a group of clinical disorders that are linked. Overlap between Sturge-Weber syndrome (SWS) and Klippel-Trenaunay Weber syndrome (KTWS) is very rarely. We presented a case of SWS with KTWS that involved upper limb. Case Presentation A 24- year-old male with extensive port-wine staining over left side of his face, extending to lower eyelids, cheeks, neck and left upper extremity. The size of mid arm circle in left side was 34 cm and in right side was 27 cm. Conclusions This is a case of overlap SWS and KTWS that involved upper limb
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