An unusual small intestinal tumor was found in a 61-year-old male who complained of intermittent right lower quadrant burning pain, tenesmus, and bloody diarrhea. On gross examination, the tumor was located intramurally in the distal jejunum with no involvement of the overlying mucosa. Histologic examination revealed two elements: a spindle cell component with characteristics reminiscent of a Schwann cell neoplasm, and nests of larger epithelioid-like cells almost undistinguishable from the Zellballen of nonchromaffin paragangliomas. The neoplasm originated within the intestinal wall without apparent mucosal origin. Ultrastructural studies revealed features recapitulating the normal enteric plexus structure and quite unlike those described in normal or neoplastic smooth muscle, Schwann or paraganglion cells. On this basis, this neoplasm is considered as a malignant enteric plexus tumor or a plexosarcoma.