A 44-year old man presented with a 2-year history of epistaxis and a nodular lesion in the nasopharyngeal mucosa. Neuroimaging revealed a midline nasopharyngeal tumor extending through the skull base to the clivus. Following surgical resection, histological studies showed a paraganglioma, a tumor with a typical nesting pattern, abundant capillary network, and strong immunoreactivity for neuroendocrine markers. Paragangliomas of the head and neck are rare tumors, which may come to the neuropathologist's attention because of their propensity for intracranial spread. In particular, paragangliomas located in the nasopharynx may generate diagnostic difficulties, as they appear to have no connection with major paraganglia in the region. Morphologic criteria do not allow distinguishing between benign and malignant forms.