OBJECTIVE: The objective of this article is to report a rare case of headache as the initial symptom of granulomatosis with polyangiitis (GPA) and to review the recent literature.BACKGROUND: Granulomatosis with polyangiitis is a rare, systemic, autoimmune disease of unknown etiology. GPA has a wide spectrum of clinical symptomatology, including involvement of the nervous system, even as the initial manifestation. Symptoms of the peripheral nervous system used to dominate the clinical symptomatology. However, recent reports are focusing increasingly in granulomatous lesions of the central nervous system, and especially on the increased frequency of patients with hypertrophic pachymeningitis (HP). We report the case of a patient with headache linked to intracranial hypertension and hypertrophic pachymeningitis as the initial and dominant presentation of GPA and we review the recent literature.METHODS: A 54-year-old male, without any related medical history developed a severe headache. In the following 2 months, he gradually developed hoarseness and diplopia at the left and lower fields of vision. A brain MRI revealed wide-spread fattening and meningeal enhancement over the left hemisphere and the left cerebellar hemisphere. An endoscopy of the pharynx revealed the presence of a tumor-like mass in the left half of the nasopharynx. A biopsy showed inflammation with presence of polykaryocyte Langhans giant cells. The laboratory testing revealed important albuminuria and microhematuria, positive c-ANCA and negative p-ANCA. A diagnosis of GPA was established.RESULTS: A steroid treatment was administered initially, which improved the headache drastically, followed by the administration of a combination of cyclophosphamide and corticosteroid, which led to a gradual resolve of the remaining symptomatology. A follow-up brain MRI showed a decrease in meningeal enhancement, whereas a second one, 2 years later, was completely normal.CONCLUSIONS: HP was considered an extremely rare manifestation of GPA. However, recent studies are reporting an increased frequency of HP and are distinguishing a granulomatous and a vasculitic phenotype, with different localization and relapse rates, that may eventually constitute a different clinical spectrum of GPA.