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Intratumoral hemorrhage/apoplexy in clival chordomas is extremely uncommon, with only 16 reported cases. The average age of patients was 46.2 years and slightly more than half were men. In cases published before 1990, all patients died from their disease without any intervention. Since then, 11 patients have undergone resection by a variety of approaches and there have been no deaths. The diagnosis of skull base chordomas relies on a combination of clinical presentation and radiographic features related to the location and invasion of the tumor. Chordomas presenting with sudden-onset symptoms should alarm the surgeon of a possible hemorrhage. As an illustration of this presentation, we describe a 58-year-old woman who presented with acute-onset headache and cranial nerve deficits. Computed tomography and magnetic resonance imaging demonstrated a hemorrhagic clival lesion with cavernous sinus extension. The patient underwent transsphenoidal resection of the lesion that resulted in the resolution of her symptoms. Histopathological evaluation of the lesion was consistent with chordoma with acute hemorrhagic components. Although intratumoral hemorrhage is rarely detected in chordomas, it should be considered a differential diagnosis of such lesions because prompt recognition and treatment are critical for patient survival.

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