Abstract  In South America the incidence of Paget’s disease of bone (PDB) is low and more than half of the cases published in the last 30 years come from Brazil and Argentina. The aim of this study was to describe the clinical and epidemiological characteristics of PDB in two institutions in Pernambuco. PDB patients in Recife, Brazil were studied retrospectively. A total of 108 cases were diagnosed from 1984 to 2005. Over 90% of the patients were of European descent. The average age was 66.2 years and 49.1% were male. The polyostotic form was the more common. Pain and bone deformity were significantly more frequent in this group and in the total group. Clear eyes were observed in 22.2% of the patients and 23.1% had a family history record of PDB. The most frequently affected bones were the pelvis, lumbar vertebrae, femurs, and skull. Zoledronate was the most effective drug in reducing the alkaline phosphatase. Our data show that PDB in Recife occurs predominantly among those of European descents, usually in the polyostotic presentation, thus providing evidence of genetic and historical factors in the prevalence of PDB in that region.