IntroductionPleomorphic adenoma is a common benign tumor of large and small salivary glands. It mainly occurs in the parotid gland, followed by the submandibular gland, sublingual gland and small salivary gland in the oral cavity. It is very rare in the nasal septum.Patient ConcernsA 27-year-old female patient attended our clinic with Nasal congestion and a diminished sense of smell.DiagnosesEndoscopic examination revealed a mass within the right nasal passage. A pathological biopsy revealed pleomorphic adenoma.InterventionsThe nasal septum pleomorphic adenoma was resected by endoscopic approach.OutcomesNo recurrence was observed for over 41 months of follow-up.ConclusionTo prevent recurrence, extensive local resection with clear histological margins and long-term endoscopic follow-up with an endoscope are necessary.