A case is described of a jugulotympanic paraganglioma in a 64-year-old woman with extentions down the jugular vein to the clavicle and penetration of the cerebellar fossa. The symptoms, differential diagnosis, therapy and prognosis are discussed on the basis of this case report and compared with the literature. The importance of radiological investigation (e.g. selective angiography and computed tomography) in respect to diagnosis and determination of tumour size is emphasized. The diagnostic value of the immunohistochemical detection of neuron-specific enolase, a neuroendocrine cell marker and S-100 protein is stressed. The most favourable therapy, depending on tumour extension, seems to comprise preoperative embolisation, radical resection and postoperative radiotherapy.

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