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Lymphangioma circumscriptum (LC) is a rare benign hamartomatous malformation of lymph vessel manifesting at birth or during early childhood. Angiokeratomas (AK) are vascular ectasia of papillary dermal blood vessels with secondary epidermal changes. AK and/or LC are one of the common vascular malformations associated with Klippel–Trenaunay syndrome (KTS). The simultaneous appearance of LC and AK has been reported rarely, and the occurrence of LC, AK, and port-wine stain in a patient of KTS is even rarer. Thereby, we report a case of co-occurrence of LC and AK in the same patient without systemic involvement.