The authors present an exceptional case of chemodectoma jugulare with important invasion of the skull base involving all the cranial nerves on one side and conforming Garcin's syndrome. Practically all the possible ways of spreading were followed by the tumor, with involvement of the posterior, middle and anterior fossas of the skull base and also orbit, middle ear and neck. The clinical and histopathological difficulties in the diagnosis, especially when the chemodectoma has only neurological signs, are stressed. The association of otologic symptoms makes the diagnosis easier. The patient had an early onset of the symptoms and a rapid evolution considering this type of tumors. The appearance of goitre in this patient is a sign of endocrine disturbance, which is occasionally associated with these tumors. Apparently this chemodectoma was not a secreting tumor, since the blood pressure values were normal. No signs of multicentricity or metastatic growths were detected. The radiological studies, and especially computerized tomography scanning, gave precise information about the spreading of the tumor. The authors consider that the knowledge of this entity is important in order to arrive at a correct diagnosis.