Ecchordosis physaliphora is a rare congenital, benign, hamartomatous, retroclival mass derived from notochordal tissue that is typically located intradurally in the prepontine cistern. Ecchordosis physaliphora is usually asymptomatic. In rare cases, ecchordosis physaliphora can be symptomatic due to tumor expansion and compression of the surrounding structures and extratumoral hemorrhage. To our knowledge, ecchordosis physaliphora associated with intratumoral hemorrhage and vasogenic edema has not been previously described. We present a case of 22-year-old man who presented with headache and confusion. MR imaging and CT revealed intracranial ecchordosis physaliphora associated with intratumoral hemorrhage and vasogenic edema. The neurological findings resolved completely after medical therapy.