We report the case of a 44-year-old woman with a one-year history of en coup de sabre morphea and progressive hemifacial atrophy with ipsilateral hemifacial neuralgia, migraine, and contralateral neurologic abnormalities. While rare, Parry-Romberg syndrome typically presents in the first or second decade of life; this patient's case is unusual in that the onset of her disease is demonstrated at age 43. Common clinical features, laboratory findings, and pathogenetic theories are discussed.