1. A case of polyostotic fibrous dysplasia with cutaneous pigmentation in a male is presented, together with the diagnostic criteria of the disorder.
2. The presence of arteriovenous communications on the side of the predominant bone involvement is advanced as additional evidence for a congenital etiology of the condition.
3. Recognition of the nature of this osseous dystrophy may prevent unnecessary surgery and possible sacrifice of normal parathyroid glands.