Generalized enchondromatosis, of which Ollier disease is the most common type, is a rare bone dysplasia characterized by multiple intraosseous tumors near growth-plate cartilage. These cartilaginous tumors have a propensity to transform into chondrosarcomas. Enchondromas of the skull base are exceedingly rare. We present the case of a patient with generalized enchondromatosis who developed a large enchondroma of the clivus, and we discuss the clinical presentation and potential treatments for this entity.
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