This case report presents a 38-year-old woman, Ucb, who exhibited a presentation of migraine-pattern headache, gradually evolving with increased intensity of the episodes and the emergence of signs and symptoms suggestive of central origin vertigo, such as axial and appendicular ataxia, multidirectional and non-fatigable nystagmus, as well as intense nausea and oscillopsia. Magnetic resonance imaging (MRI) revealed leptomeningeal enhancement in the cerebellum, brainstem, and cervical spinal cord. Analysis of cerebrospinal fluid indicated the presence of pleocytosis and hyperproteinorrachia. After excluding other systemic disease foci, a meningeal biopsy was performed, which subsequently confirmed the diagnosis of neurosarcoidosis. Early diagnosis allowed for the initiation of corticosteroid therapy, resulting in significant improvement in symptoms, while the imaging changes on MRI persisted for several months. This report emphasizes that the phenotype observed in neurosarcoidosis with leptomeningeal involvement may initially not be distinguishable from the onset of a migraine episode.