Ecchordosis physaliphora is a rare hamartomatous lesion of notochord origin that is usually asymptomatic. In this case report, such a lesion arose at the clivus, with extension through to the sphenoid sinus. As a result, it presented with cerebrospinal fluid rhinorhea, making the case particularly unusual. We describe the treatment for this rare situation and discuss its differentiation from chordoma, its malignant counterpart.
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