This case series presents the neurological complications of Rosai-Dorfman disease in three patients, each with distinct clinical presentations that illustrate a wide range of potential effects of the disease. The objective of this series is to highlight the differential manifestations of the disease including immune-related and widely disseminated disease.

We reviewed the quality control database of the Brain Tumor Center at our institution for patients with RDD. Only those with primary neurological manifestations and histopathologic confirmation were included. We recorded demographics, diagnostic data (including histopathology and neuroimaging), treatment, and outcomes.

Three cases were identified. The first patient had cervical lymphadenopathy and a right submandibular mass, along with a homogeneously enhancing extra-axial lesion initially suspected to be a meningioma. The second patient presented with ischemic infarcts and a right internal carotid artery thrombus, likely related to antiphospholipid syndrome, which may correlate with immune-related RDD and possible autoimmune lymphoproliferative syndrome. The third patient exhibited widely disseminated disease, including multiple extra-axial masses, calvarial, clival, and spinal lesions.