Unusual progression of Rosai-Dorfman disease in a pediatric patient

Rosai-Dorfman disease is a rare and self-limiting disease process that presents most commonly in young patients as massive, painless, cervical lymphadenopathy. CNS is a rare site for this disease, and only a few imaging appearances have been described. We report an unusual presentation of Rosai-Dorfman disease with cervical lymphadenopathy and involvement of the paranasal sinuses, intra and extracranial portions of the third division of the right trigeminal nerve, lacrimal glands and subsequent involvement of intra and extracranial portions of the third division of the left trigeminal nerve. Bilateral involvement of the trigeminal nerve with Rosai-Dorfman disease is rare and has only been described in 1 prior case. This case highlights that Rosai-Dorfman disease should be considered in pediatric patients with a mass involving the trigeminal nerve and extensive paranasal sinus disease.