The diagnosis of CL can only be confirmed by histological analyses and is based on well-established criteria.[6] Those include a well circumscribed cystic lesion with or without endothelial lining, a stroma characterized by meshwork of collagen and fibrous tissue and a wall containing focal aggregates of lymphoid tissue.[10] Lymphatic vessel endothelial receptor-1, vascular endothelial growth factor-3, monoclonal antibody D2-40 and prox-1 are used as immunohistochemical markers in the diagnosis of lymphangioma.[11]. This evidence concerns the gene PROX1 and lymphangioma.