Introduction . Progressive facial hemiatrophy (Parry-Romberg syndrome) is a relatively rare disease of unclear aetiology. Some authors postulate its relation with linear scleroderma.

Objective . To present 2 cases: facial hemiatrophy and linear scleroderma localized on the head with different clinical picture.

Case report . We present two cases: a case of a 49-year-old woman with a typical picture of hemifacial atrophy, without any changes in the central nervous system; and a case of a 33-year-old patient with scleroderma en coup de sabre and extracerebral tumour. In a patient diagnosed with Perry-Romberg syndrome, with Borrelia burgdorferi infection and minor neurological symptoms, for the 4-year duration of the disease the proper diagnosis and therapy have not been established. In the second patient only skin changes without any neurological symptoms were observed, but through investigations of the central nervous system revealed the presence of an extracerebral tumour.

Conclusions . The presented cases, especially the second one, indicate the necessity of detailed neurological examination in patients with progressive facial hemiatrophy and scleroderma en coup de sabre in spite of a lack of subjective complaints.