Epithelioid vascular tumors encompass a spectrum of diseases that includes epithelioid hemangioma (EH), a benign neoplasm, epithelioid hemangioendothelioma (EHE), a low to intermediate grade malignancy, and epithelioid angiosarcoma (EAS), a high grade malignancy. Although certain morphologic features allow to distinguish EHE from EH and EAS, the diagnosis can be challenging due to considerable morphologic overlap, particularly on small biopsies or when EAS lacks vasoformative properties. Clinical behavior and, consequently, treatment and prognosis vary significantly among vascular tumors. Therefore, it is paramount to effectively distinguish them from each other. The recurrent translocation t(1;3) (p36.3;q25.1) was identified in approximately 90% of EHE cases, but not in other vascular tumors. t(1;3) results in the WWTR1-CAMTA1 fusion gene which encodes a putative chimeric transcription factor which is under the transcriptional control of the WWTR1 promoter. A recurrent YAP1-TFE3 gene fusion has been identified in WWTR1-CAMTA1 negative EHEs. Thus, FISH analysis for the presence of WWTR1 translocation may serve as a useful molecular tool in the differential diagnosis of challenging cases.