14 patients were included (median age 38; female 9/14; Black 13/14) and diagnostically supported by systemic (11/14) and neurologic (3/14) biopsies. Systemic involvement was present in all patients and commonly included the hilar lymph nodes (12/14), pulmonary parenchyma (8/14), and sinuses (5/14). Patients typically presented chronically (median delay 75 days) with concurrent neurologic and systemic sarcoidosis (9/14) and hypothalamic-pituitary involvement as the inaugural manifestation (7/14). Headache (12/14), memory deficits (9/14), polyuria (8/14), polydipsia (7/14), vision impairment (7/14), and temperature dysregulation (6/14) were common complaints. Of those with MRI at symptom onset (12/12), abnormalities were seen in the hypothalamus (enhancement 12/12, expansile mass 8/12), infundibulum (thickening 12/12, enhancement 11/12), and pituitary (7/12 both anterior and posterior pituitary, 2/12 suprasellar extension). Adjacent structures (leptomeninges 10/12, optic chiasm 7/12, mammillary bodies 8/12) were commonly involved, and almost all (11/12) had non-hypothalamic-pituitary cranial neuroinflammation. Elevated serum ACE and CSF WBC were present in 5/11 and 10/11 patients, respectively. After a median follow-up of 66 months, the median EDSS was 6.25, all patients with HPA dysfunction (12/14) remained on hormone replacement and/or glucocorticoids, 11/14 patients relapsed, and a median of 2 (range 0-5) steroid-sparing immunosuppressants were utilized.