A recurrent translocation t(1;3)(p36.23;q25.1), resulting in the fusion of CAMTA1 on 1p36.23 to WWTR1 on 3q25.1, has recently been identified in most EHE, even if in different sites and grades of malignancy [21,22]; this translocation has not been detected in other pathologies like epithelioid hemangioma, epithelioid angiosarcoma or pseudomyogenic (epithelioid sarcoma-like) HE, which often mimic EHE and make diagnosis more difficult. Here, CAMTA1 is linked to epithelioid hemangioendothelioma.