BackgroundCutaneous lymphatic malformations represent a group of heterogeneous diseases caused by developmental defects of lymphatic system.ObjectiveThe purpose of this study was to report the clinical, histopathological and immunohistochemical features of a distinctive lymphatic malformation.MethodsTwelve patients with similar clinical and histopathological features were included in this study. Immunohistochemical staining of CD31, D2‐40, Prox1 and Wilms tumor 1 (WT‐1) were performed on all lesions.ResultsAll cases were either congenital lesions or developed during the first 2 years of life. All presented as red to brown papules or nodules on acral sites. Histopathologically, the lesions consisted of a dermal proliferation of flat or slit‐like vessels lined with a single layer of endothelial cells. Hemosiderin or extravascular red blood cells were present in all cases. The constituent vessels expressed CD31, D2‐40 and Prox1 and lacked expression of WT‐1.ConclusionOn the basis of the clinical, histopathological and immunohistochemical findings, our cases represent a unique type of lymphatic malformation that we believe is distinct from previously reported vascular proliferations. We propose the name of acral hemosideric lymphatic malformation for this entity.