ABSTRACT:We report a case of a 59‐year‐old male with a history of hypertension, polycythemia, and optic neuritis, who presented with progressive visual loss, bilateral lower extremity weakness, and new‐onset urinary and fecal incontinence. MRI findings of leptomeningeal enhancements raised suspicion for neurosarcoidosis, leading to initial treatment with intravenous immunoglobulin (IVIG), but with no significant improvement. Six weeks later, the patient developed an acute ischemic stroke, confirmed by MRI, alongside worsening neurological deficits. Further evaluation revealed mediastinal and hilar lymphadenopathy on chest CT, supporting the diagnosis of neurosarcoidosis. Despite a course of high‐dose methylprednisolone and immunosuppressive therapy, including mycophenolate mofetil, the patient showed partial recovery in strength but only minimal improvement in vision. At follow‐up, MRI demonstrated stable disease with new leptomeningeal enhancement, prompting the initiation of TNF‐alpha inhibitors. This case underscores the rare but important association between neurosarcoidosis and ischemic stroke, highlighting the complexity of diagnosis and management, which includes both stroke prevention and sarcoidosis treatment.