Parry-Romberg syndrome (PRS) is a rare disorder of uncertain etiology that is characterized by progressive atrophy of the soft and hard tissues of face, typically occurring in the first 2 decades of life. It is more commonly seen in females. The disease progresses slowly with gradual atrophy, frequently associated with neurological, ophthalmological, and other system involvement, resulting in secondary complications. The severity of deformity varies depending on the age of onset of disease. Those in whom the disease starts at an earlier age will have more severe deformity. Due to the visible facial deformity, such patients usually suffer from social and psychological trauma. Management is mainly cosmetic, which is carried out after disease progression has stopped and stabilized. This brief review describes PRS in detail and compares it with linear morphea en coup de sabre (ECDS), its close differential, which is likely to be a milder variant sharing the same spectrum of disease.