A 56-year-old woman with vertex headaches, new-onset seizure disorder, nasal obstruction, and hyposmia was found to have a dural-based intracranial mass as well as bilateral masses arising from the inferior turbinates and extending into the nasopharynx. The patient underwent endoscopic resection of the involved portions of the inferior turbinates, including the sizable nasopharyngeal component. Craniotomy was performed at a later date. Both pathologic specimens were consistent with extranodal Rosai-Dorfman disease. The patient showed resolution of all symptoms after surgery. Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare entity usually presenting as cervical nodal disease. When extranodal disease of the head and neck is present, prompt recognition and conservative surgical management is the treatment of choice for this benign pseudolymphomatous entity.