A patient with a clinical and serological diagnosis of CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal motility abnormalities, sclerodactyly, telangiectatic lesions), a variant of PSS (progressive systemic sclerosis), was found to have multiple intracranial aneurysms. She subsequently developed bilateral optic neuropathies. Ischemic and compressive etiologies are proposed and related to the histopathology of the patient's underlying systemic disease.