This is a report of a 28-year-old HIV+ man with extensive involvement of lungs, spinal roots and peripheral nerves by neurofibromatosis von Recklinghausen. Huge masses (neurofibromas) ventral of the spine, which already infiltrated into the foramina intervertebralia, could be located by magnetic resonance imaging and high resolution computer tomography. Strikingly, most biopsies taken by bronchoscopy of the bronchial wall from various parts of the airways revealed extended infiltration by neurofibromas in submucosal areas, but no malignant tissue components. In addition, HIV infection with low CD4/CD8 ratio (0.1) and low CD4+ cells (160/microliters) was found.