Introduction: Parry-Romberg syndrome, a rare syndrome, is characterized by progressive atrophy affecting one side of the face. This disease can include the skin, subcutaneous tissue, muscles, cartilage, and underlying bony structures.

Case Presentation: A 27-year-old female was admitted to the outpatient clinic of dermatology of Farshchian Hospital, with chief complaint of asymmetric facial appearance. The asymmetry started at the age of 16 and developed over a period of 4 years, then stopped. A physical examination revealed that the right side of her face, especially her chin was smaller than the left side, and there was loss of subcutaneous fat, which was associated with skin tightness. There were no systemic symptoms of other illnesses or other signs.

Conclusion: The disease is self-limiting, yet, can be associated with systemic involvement such as neurological disorders. Clinical symptoms are the keys of diagnosis. A muli-lateral approach is essential to control the disease.