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Migraine‐like associated with chest pain is an alarming association and forces us to rule out the presence of a secondary cause. That must be taken into account in the differential diagnosis of craniofacial hemicranial pain that appears in patients with no personal history of headache, and risk factors for the development of pulmonary neoplasia.

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Isolated, space-occupying lesions in the brain encompass a wide differential diagnosis. Autoimmune diseases may be at the origin of these lesions and should be included in the diagnostic process. Isolated brain involvement by Sjögren's syndrome is very rare. We present the first case published in the literature confirmed with brain biopsy, secondary to a cerebellar pseudotumoral lesion due to localized lymphocytic vasculitis as the first manifestation of a Sjögren's Syndrome.