Paraganglioma is a rare tumor in the upper respiratory tract. More than 30 paragangliomas have been reported arising in the larynx but only a few in the trachea. The organoid arrangements of the cell nests reproduce the classic ‘Zellballen’ of the normal gland. Neurosecretory granules can be seen at electron microscopy. Angiography may be a valuable diagnostic adjunct. Malignant degeneration may occur. The 44-year-old woman reported had a dumbbell-shaped tumor involving the subglottic larynx but with its largest portion outside the larynx and within the left lobe of the thyroid gland. The tumor may very well have arisen from the inferior laryngeal paraganglia. An important differential diagnosis is hemangiopericytoma, which was the preoperative diagnosis in this case. A laryngectomy including the left lobe of the thyroid gland was performed and there is no sign of local recurrence or metastases 7 years after operation.

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