We present a case of unusual clinical picture of the well defined Morbus Von Recklinghausen. In a 35-year-old male patient, after an uncontrolled rapid movement of the neck, the disease was suddenly manifested with spastic quadriparesis. Three years later the clinical picture showed spastic quadriplegia. Antalgic torticollis confirmed by X-ray of the neck clearly showed vertebral subluxation at the level of C3-C4. However, additional magnetic resonance imaging revealed large extradural tumorous formation at the level of C1-C2, and two small oval tumours in the splenium of the corpus callosum as well as in the left lateral ventricle. "Café au lait" spots, "molluscum fibrosum" and few subcutaneous fibromas appeared as skin changes which explained the possible nature of the registered intracranial and intraspinal tumours. Neurosurgery has been performed at the spinal level with good results; it confirmed the diagnosis of Von Recklinghausen's Neurofibromatosis.