Parry-Romberg syndrome is an orphan disease characterized by slowly progressive unilateral atrophy and degeneration of the face skin, soft tissues and bones with deformity and asymmetry of the face. In some cases, Parry—Romberg syndrome is combined with linear scleroderma as a saber strike. The association between these two rare conditions is still unclear. These diseases have clear clinical and morphological differences but their frequent combination may indicate common pathogenesis mechanisms. Localization of pathological foci on the face may be accompanied by a decrease in the quality of life due to the presence of a cosmetic defect that leads to the depression and anxiety. Late diagnosis and inadequate therapy can lead to disability and social maladjustment of patients. We present a review of modern literature and a clinical case of a rare neurocutaneous syndrome — Parry—Romberg disease in combination with linear scleroderma (en coup de sabre) in a woman aged 24 years. Raising the awareness of dermatovenerologists, neurologists, otorhinolaryngologists and doctors of other specialties will make it possible to diagnose a combination of two diseases in a timely manner and provide adequate therapy.