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Primary vascular tumors of the lymph nodes other than Kaposi's sarcoma are rare. The present study describes the case of a primary hemangioendothelioma in a cervical lymph node in a patient treated for a carcinoma of the large bowel. Microscopically, the structure of the lymph node was completely substituted by tumoral proliferation, with an architectural pattern consisting of anastomosing vascular channels, a number of which contained papillary projections or a tuft-like structure. This study demonstrates the potential diagnostic errors and hypothesizes the pathogenesis of this type of lesion; a vascular endothelial growth factor (VEGF)/VEGF receptor-3 (VEGFR-3) autocrine loop may be activated.