Aims and background: Primary paragangliomas of nasal cavity and paranasal sinus are rare conditions that could show aggressive behavior with local recurrence and metastasis development. The diagnosis of malignancy is challenging because there are no available histopathologic criteria. In these cases, the prognosis is usually poor.
Methods: We report a case of a woman with malignant paranasal sinus paragangliomas.
Results: The patient was treated with several surgical approaches, radiotherapy, and medical therapy for different recurrences.
Conclusions: Despite the malignant behavior of the disease, this patient is still alive many years after first diagnosis.