Background: Rosai-Dorfman disease (RDD) is a rare non-Langerhans histiocytic disorder with less than 5% central nervous system involvement and is often mistaken for meningioma given the similarity in imaging features. The authors present the unique case of a 44-year-old female who presented with ongoing visual impairment.
Observations: A purely suprasellar mass was noted on magnetic resonance imaging and was initially diagnosed as craniopharyngioma. Unexpectedly, the pathology report revealed RDD.
Lessons: To date, only six cases of sellar RDD have been reported, and our case is the first reported with a purely suprasellar presentation. No standard treatment has been established for RDD, and next-generation sequencing may be a promising therapeutic option.
Keywords: Rosai-Dorfman disease; central nervous system involvement; craniopharyngioma; histiocytosis; suprasellar tumor.