Paragangliomas are common in carotid body, jugular tympanic cavity and cervical vagus nerve, but uncommon in larynx. We report clinical and pathological features of a 40-year-old woman with laryngeal paraganglioma. Tracheotomy combined with vein anesthesia was performed to isolate tumors. The patient was followed up for 3 months after surgery treatment and recovered well. We also provide a better understanding of this disease through a review of relevant literature. Largnx paraganglioma is rare, but should require long-term follow-up for possible recurrence.