A patient presented with an atypical left lateral medullary syndrome (LMS) mimicking a stroke. Magnetic resonance imaging demonstrated a tumour in the left dorsolateral part of the medulla. Biopsy revealed a grade II astrocytoma. The patient died two weeks after surgery. Nine pathologically verified previous cases of LMS caused by a tumour are reviewed. LMS is invariably atypical because of associated supratentorial or extensive brainstem symptomatology. Onset is usually progressive but stroke-like onset has been occasionally reported. Glial tumours as a cause of LMS are less frequent than metastasis. Successful treatment has been reported in a case of low grade astrocytoma.