A 54-year-old female with a past medical history of systemic arterial hypertension, presented with hemicrania occipital oppressive headache, associated with numbness in both arms and legs, and progressive gait instability. Her initial evaluation was elsewhere and included magnetic resonance imagining (MRI) that reported a mass lesion in the inferior clival region surrounding the right vertebral artery. Embolization of the right vertebral artery was performed, and the patient was referred to the out-patient clinic. She would reach our hospital three months later. of the soft palate was detected as well as peripheral facial palsy, House Brackman III. Later on, she was diagnosed with diabetes insipidus, rare given the mass location. Postoperative Computed Tomography (CT) scan showed no evidence of haemorrhage or acute hydrocephalus. Pathology reported a xanthogranulomatous lesion without evidence of malignancy. Immunohistochemistry showed focal reactivity to S100, foamy histiocytic infiltrates positive for CD68 and negative for CD1a as well as CD45, CD3 and CD20 lymphocytic infiltrates. Neurosarcoidosis was still being considered, also the possibility of mycobacterial infection was contemplated given our regional epidemiology and IgG4 disease. The ratio of plasma cells IgG4/IgG was not greater than 40% not meeting diagnosis criteria. Chest CT showed a nodular pericardial thickening with periaortic inflammatory tissue indicative of periaortitis, pericardial and pleural effusion.