Photophobia is a common neurological and ophthalmological symptom that has been associated with a growing number of neurosurgical conditions, especially compressive lesions. The exact signaling pathways and neurophysiological features of the disorder are not well understood; however, data from multiple studies have shown the significance of the trigeminal system and the pretectal nuclei in its pathophysiology. The authors report on a rare case of a blind patient who presented with photophobia without evidence of light perception. They also review the literature and early experimental data in an effort to understand the possible neuronal pathways and structures involved in photophobia.