The medical records of 64 patients with paraganglioma of the head and neck seen between 1954 and 1984 at the University of Texas M.D. Anderson Hospital, Houston, were reviewed. Lesions were found in the carotid body (n = 24), jugulotympanic area (n = 37), vagal nerve (n = 4), and larynx (n = 1). A hereditary pattern was identified in six patients (9%). Metastases developed and caused the death of two patients (8%). No functional secreting tumors were identified. Paragangliomas are often misdiagnosed, both clinically and pathologically. Angiography is the definitive clinical study for diagnosis and treatment planning. Computed tomographic scanning further defines extent of the disease. Lesions in the neck are effectively treated by surgery. Jugulotympanic tumors can be successfully managed by surgery, radiation therapy, or a combination of both depending on the size and extent of the tumor. Secondary treatment of residual disease can be as effective as primary treatment.