The epithelioid hemangioendothelioma of the liver has been recently characterized as a rare tumor with distinctive pathological features affecting young adults. Our report describes a case of histologically confirmed primary epithelioid hemangioendothelioma of the liver, diagnosed by clinical examination as well as radiological (CT/MR) and scintigraphic imaging (labelled red cells/ phytate-SPECT). This case highlights the usefulness of nuclear medicine techniques during the diagnostic of this rare tumor. Further it stresses the possibility of employing an easy and noninvasive method to adequately follow-up those patients who cannot be considered as disease-free even after orthotopic liver transplantation because of the recurrence of the disease in the transplanted liver.