Spontaneous intracranial hypotension is characterized by postural headache that is generally associated with neck and/or back pain, radicular symptoms, nausea, and vomiting, and can sometimes be accompanied by cranial nerve symptoms. Although ocular manifestations are common, visual impairment due to optic nerve involvement is not commonly reported. Here, we report a case of a 34-year-old woman with postural headache and visual loss in the left eye associated with spontaneous intracranial hypotension. The orbital magnetic resonance imaging revealed increased intensity on T2-weighted images and on the pathologic contrast enhancement of the left optic nerve. Radionuclide cisternography showed bilateral cerebrospinal fluid leakage at the top of the thoracic region. To our knowledge, this is the first report documenting an association between optic neuropathy and spontaneous intracranial hypotension.