Progressive facial hemiatrophy or Parry-Romberg syndrome is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue, and the underlying bony structures. This syndrome has many features of linear scleroderma en coup de sabre but is distinguished by more extensive involvement of the lower face and by only slight cutaneous sclerosis. We describe two unusual children with both atrophic and sclerotic changes of half of the face coexisting with multiple plaques of typical morphea. Both children developed neurologic disturbances with cranial magnetic resonance imaging (MRI) abnormalities 2 years and 15 years, respectively, after the onset of cutaneous lesions. Thus considering that it may not be possible to correlate impairment in neurologic function and cutaneous disease, as illustrated by our patients, we emphasize the importance of an accurate follow-up.