Parry-Romberg syndrome or progressive facial hemiatrophy (PFH) is a rare disease of unknown etiology characterized by atrophy of the skin and subcutaneous tissue on one side of the face. The authors present the case of a 32-year-old woman with PFH who had migraine and an intracranial aneurysm. The findings support the hypothesis that the disease could be related to a neural crest migration disorder, from which both fronto-nasal mass and cranial vessels take origin.