Collet-Sicard is a rare syndrome that consists of the palsy of all the lower four cranial nerves. We describe this entity in relation with a schwannoma of the hypoglossal nerve. A 45 year-old-patient was admitted to the hospital referring hoarseness and difficulty in swallowing for two weeks. On neurological examination, the patient exhibited palsy of the ninth, tenth, eleventh and twelfth nerves. This is a syndrome of Collet-Sicard. The cranial MRI revealed a small intracranial and extracranial tumor adjacent to the hypoglossal foramen. The tumor involved the jugular foramen and was moderately enhanced with gadolinium The patient underwent surgical removal. The pathologic examination of the surgical specimen confirmed the diagnosis of a schwannoma of the hypoglossal nerve. We have carried out a research of the Collet-Sicard syndrome and of its aetiology. Although schwannoma the hypoglossal nerve is a rare disorder we consider that this entity should be included in the differential diagnosis of the Collet-Sicard syndrome.