Parry-Romberg syndrome is an acquired slowly progressive disease characterized by an atrophy mostly involving half of the face. The pathogenesis of this disfiguring condition is still controversial. The relationship between Parry-Romberg syndrome and Lyme disease needs to be considered in depth. A 16-year-old woman from Albania presented with linear depressions of the right side of the face, clinically compatible with Parry-Romberg syndrome. She had a positive history of Lyme disease. Borrelia infection was confirmed by the positivity of PCR and the presence of IgM antibodies. The patient received intravenous penicillin and metronidazole for 14 days. After treatment and during a 2-year follow-up, the clinical disease progression was halted and the serological and microbiological tests for Borrelia burgdorferi sensu lato were negative. We cannot exclude a coincidence, however, of the bacteriological and serological evidence. Moreover, the interruption of the disease progression after the antibiotic therapy is difficult to ignore without claiming that this association is at least suggestive.