The neurogenic sarcoma is a neoplasm found seldom. It descends from the sheaths of peripheric nerves. If this tumor develops from a solitary or multiple neurofibroma, it might also be called a neurofibrosarcoma. All other synonyms, especially that of the malignant schwannoma, should better be abandoned for histogenetic reasons. Today the classification of all tumors of the peripheral nervous system is based on a suggestion of the WHO from 1969. The tabular summary of the literature shows that only a little more than a hundred cases of neurogenic sarcoma of the head and neck were reported during the last 50 years. Differential diagnosis of this malignant tumor includes nearly all other malignant neoplasms of soft tissue, sometimes even anaplastic carcinoma. Histopathology, including electron microscopy, is described in general and also in detail with regard to three cases. Three forms of micro-morphological variants are dealt with, too. For diagnosis histological examination will be absolutely successful only in cases of neurofibromatosis or in cases where the nerve from which the tumor originates may be identified. Otherwise, conventional and computerized X-ray tomography may be helpful. Finally, some therapeutic and prognostic principles are discussed.

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