We observed a 42-year-old woman presenting with severe dysphagia secondary to paralysis of the lower cranial nerves and right phrenic nerve involvement, followed by respiratory failure. An EMG confirmed bilateral denervation of the 9th, 10th, 11th and 12th cranial nerves and right phrenic nerve. Videolaryngoscopy showed bilateral vocal fold immobility. Anemia, elevated ESR, microhematuria and C-ANCA (PR-3) antibodies were detected. Brain MRI and CSF were normal. A chest CT showed bilateral, irregular pulmonary lesions. An 18F-FDG total body scan showed diffuse hypermetabolic regions in both pulmonary bases, in the mediastinic region and in the rhinopharynx, raising the suspicion of a neoplastic process. A transthoracic biopsy disclosed nodular granulomatous aggregates with multinucleated giant cells, supporting the diagnosis of Wegener's granulomatosis. Immunosuppressive therapy achieved complete clinical resolution and cleared the pulmonary lesions. To the best of our knowledge this is the first report of Wegener's granulomatosis presenting with neurogenic dysphagia due to lower cranial nerve palsy.