Parry-Romberg syndrome (PRS) is an uncommon degenerative condition characterized by a slow, progressive, and, generally, unilateral atrophy of facial tissues, including muscles, bones and skin. Ophthalmological and neurological manifestations have frequently been observed and few oral changes have been reported. This article reports a case of PRS in a 22-year-old woman, exhibiting facial asymmetry, hypoplasia of the right side of the face, areas of skin hyperpigmentation, and oral alterations, involving the mandible and teeth. These clinical and radiological findings led to the diagnosis of PRS. In an attempt to improve the patient's facial aesthetic and the dental functions, oral pentoxifylline, orthodontic rehabilitation, and subcutaneous injections of polymethylmethacrylate microspheres were used as part of the treatment for the facial atrophy. Together, these approaches accounted for a minimal invasive treatment with long term satisfactory results.