OBJECTIVE: The objective of this study is to report a case of Rosai-Dorfman disease (RDD), which is a rare condition that involves the neck and head region. CASE REPORT: A 58-year-old woman reported symptoms starting 6 years prior with nasal obstruction and a reduced sense of smell and taste. The endoscopic exam showed a bilateral nasal mass. She reported that the nasal obstruction had worsened, with nasal discharge and headache. CT revealed a bilateral nasal mass and pansinusitis. Surgery was performed, improving nasal patency. An anatomopathologic exam of the polyps revealed histiocytosis and was positive for the S-100 protein, which indicated a diagnosis of RDD. RDD is a histiocytosis, is most common in young people, and is accompanied by lymphadenopathy, generally cervical and painless. A third of these patients have extranodal manifestations, 75% in the head and neck region. In this case, the patient was a 58-year-old with a nasal mass. The treatment of this condition can involve corticosteroids, radiotherapy, and chemotherapy with inconsistent results in the literature. Recurrence is seen in a majority of cases. At the moment, the patient is utilizing a topical corticosteroid, without nasal, systemic, or nodal manifestations. CONCLUSION: Rosai-Dorfman disease is a rare, benign disease that can cause sinus manifestations, and surgery in addition to topical corticosteroids can maintain good control of the symptoms.