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Objectives: To describe the typical presentation, pathology findings, and treatment options for patients presenting with Rosai-Dorfman disease. Study Design: This case report was completed using chart review for data collection. Methods: The case report was generated through chart review looking at physical findings found at presentation, intra-operative findings, and pathology. A literature search was completed, investigating typical presenting symptoms, surgical management, pathology findings, and treatment options. Results: The patient was a 38 year old gentleman who presented with a six month history of nasal congestion and recurrent epistaxis. He was found to have significant enlargement of the nasal septum with no cervical lymphadenopathy. Surgical excision of the lesion via open rhinoplasty approach revealed surgical pathology consistent with Rosai-Dorfman disease. Due to the lack of involvement of other head and neck sites, no other intervention was necessary but close observation was recommended. Conclusion: Rosai-Dorfman disease can present with isolated head and neck extranodal involvement at sites including the nasal septum. While no ideal treatment has been determined, interventions should correspond to the degree of symptoms experienced. Adjuvant therapies may be indicated beyond surgical excision. [ABSTRACT FROM AUTHOR]