Primary epithelioid haemangioendotheiomas (EHE) of the inferior vena cava (IVC) are extremely rare malignant tumours of vascular origin. Only about 40 case of vascular EHEs are described in the literature, and only fourorigin by IVC (1-4) and two by superior cava vein (5,6). Only surgical therapy for EHE has proven beneficial.It is often difficult to make the right diagnosis with imaging before histhological examination. Imaging, usually, help to surgical planning, but in our case helps to make the right diagnosis. We present a rare case of a patient with an IVC EHE, studied with Computed Tomography Angiography (CTA), Magnetic Resonance (MR), and Positron EmissionTomography Computed Tomography with fluorine 18 fluorodeoxyglucose (18F-FDG PET/CT).