Neoplastic angioendotheliosis (NAE) is a rare neoplastic disease, and its pre-mortem diagnosis is extremely difficult. A 49-year-old male developed vertigo, hearing and visual disturbance, transverse myelopathy below Th 5 and hypercalcemia. These symptoms were markedly improved by VEPA chemotherapy. Thirty-four months after onset, diffuse reticular shadows were noted on chest X-ray. The biopsy specimen of the lung revealed intravascular lymphoid cells differentially stained with L26 and LCA. Southern blot analysis of the DNA from the tissue showed rearranged bands for the immunoglobulin gene (JH). A diagnosis of NAE of B-cell nature was established.