To report a case of prosopometamorphopsia in a patient with right occipital subacute ischemic stroke and review the anatomical basis and pathophysiology of this extremely rare visual perception disorder characterized by diverse facial distortions.

Prosopometamorphopsia is one of the 40-odd types of metamorphopsia seen in Alice in Wonderland syndrome. Any part of the face-processing network can be involved, from the occipital to frontal lobe, with a key role of the corpus callosum due to interhemispheric transfer. Causes are numerous including stroke, seizure and migraine. It can affect the perception of the whole face, particularly in right or bioccipital lesions, while hemi-prosopometamorphopsia appears to be related to ipsilateral lesions to the distorted hemifield or to lesions of the splenium of corpus callosum. This may cause significant distress to patient and impair rehabilitation in case of stroke. Fortunately, it is mostly transient and self-limited due to high adaptability of the network.

Prosopometamorphopsia, though extremely rare, can create significant distress and impair stroke rehabilitation, but is usually a self-limited phenomenon.