A patient with a large ecchordosis physaliphora extending from the sphenoid sinus into the subarachnoid space of the prepontine cistern and resulting in a cerebrospinal fluid fistula is described. Ecchordoses are most commonly asymptomatic and found only incidently at autopsy. This case report adds to the scant literature on symptomatic ecchordoses. The previously reported cases of symptomatic ecchordoses and intradural chordomas are briefly reviewed. Differentiation of chordoma and symptomatic ecchordosis may be difficult; however, the intradural location and relatively benign behavior of the latter are useful points. A discussion concerning the remnants of the notochord which persist in the adult and their role in the genesis of chordoma and ecchordosis physaliphora is also provided.