The classification of certain vascular bone tumors that show an epithelioid cytologic appearance remains confusing, with overlap in features of epithelioid hemangioma, hemangioendothelioma and epithelioid hemangioendothelioma. Radiographs of a 27-year-old woman who presented with ankle pain showed an expanded lytic-sclerotic lesion in the distal left fibula. Magnetic resonance imaging showed an intramedullary lesion with a small lateral intracortical component. The lesion was hypo- to isointense to muscle on T1-weighted images and heterogeneously hyperintense on T2-weighted images. Initial incisional biopsy was inconclusive. Open biopsy showed hemangioendothelioma with epithelioid morphology, and the lesion was completely resected with reconstruction using a peroneal fibular rotation graft. Examination of the resected specimen showed focal hemangioendothelioma with an epithelioid phenotype arising in a hemangioma. This case illustrates the difficulty and pitfalls of making the correct diagnosis on the basis of a small biopsy specimen.