A case of dumbbell-shaped hypoglossal neurinoma with intra- and extracranial extension is reported. The tumour was surgically completely removed in a one-stage operation via a dorsolateral sub-occipital transcondylar approach. Clinical presentation and the role of high-resolution CT-scan, MRI and angio-MRI in diagnosis and surgical planning are discussed. We include a review of the literature concerning these rare tumours of the foramen magnum region.
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