
Parry Romberg Syndrome (PRS) is relatively rare condition of debatable etiology, usually restricted to one side of the face, rarely being bilateral or involving the limbs. Characteristically, the atrophy progresses slowly for several years and become stable after certain time period. After stabilization of the disease multispeciality approach including physicians, dental surgeons, psychologists and reconstructive surgeries can be performed to correct the deformity. PRS is frequently associated with localized scleroderma, known as “en coupe de sabre” (ECDS). A debate exists whether PRS is a form of linear scleroderma or these conditions are inherently different processes or appear on a spectrum. We present a case of progressive hemifacial atrophy of left side of face in a 28 year old male with ECDS without any neurological or ophthalmic complications.