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The syndrome of multiple enchondromas is known as Ollier's disease. Enchondromas are benign tumours of hyaline cartilage arising within the medullary cavity of tubular bones. We are reporting the case of a 16 year old girl with Ollier's disease who presented with seizures and brainstem compression. A MRI scan of brain showed an intracranial space-occupying lesion in the region of clivus. The intracranial tumour was surgically removed and the histopathology confirmed the diagnosis of enchondroma. Intracranial enchondroma is an extremely rare situation and reported for the first time from south India.