PURPOSE: To present two patients in whom photophobia was a predominant presenting symptom of retrochiasmal demyelination. DESIGN: Observational case report. METHODS: Two women who experienced an acute onset of photophobia underwent neuroophthalmic examination and investigations at a single institution. RESULTS: Examination revealed a homonymous visual field defect in both patients. Magnetic resonance imaging (MRI) demonstrated a hyperintense lesion in the area of the right posterior thalamus in one patient and enhancing lesions in the deep white matter of the temporal lobes bilaterally with contrast enhancement of the right optic tract in the second patient. Additional investigations, including lesion biopsy in the first patient, revealed demyelination as the most likely etiology. Improvement of photophobia, visual field defect, and radiographic abnormalities was documented over the next three to four months without accompanying treatment in both patients. CONCLUSIONS: Central pathology, including demyelination of the retrochiasmal visual pathway, should be considered in patients who experience acute photophobia.