Introduction: Adenoid cystic carcinoma is a malignant tumor, representing not more than 1% of all head and neck cancers. This is an epithelial neoplasm usually arising from salivary glands. On rare occasions, it may be reported to arise from the lateral wall of the nasal cavity. Case report: This is a case report of a 79-year-old female presenting with progressive bilateral nasal obstruction, complete obstruction of the Rt nasal airway, and partial obstruction of the left nasal airway over the past six months, with no pain or epistaxis. A biopsy confirmed the diagnosis of adenoid cystic carcinoma. Endoscopic resection assisted by Coblator II was performed. Conclusions: Adenoid cystic carcinoma of the nasal septum is rare but requires careful diagnosis and a tailored treatment approach. Long-term follow-up is essential due to the risk of recurrence and metastasis. Early detection and comprehensive management are key to improving outcomes for patients with this unusual presentation.
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