Although it is fairly common in the Western countries, Paget's disease of bone is very rare in Chinese individuals. Through an extensive literature search, only 8 Chinese Paget's disease cases were found in the English language literature and all were reported in local medical journals of Asia that may not be accessible to international readers. To enhance the awareness of the rarity of Paget's disease in Chinese individuals to pathologists worldwide, we present a case of Paget's disease in a 54-year-old Chinese male. We also compare the clinical features of Paget's disease reported in Chinese patients to Paget's disease reported in the Western countries. No familial cases and no malignant transformation are found in the reported cases of Paget's disease in Chinese patients. In addition, more often skull involvement, higher frequency of monostotic cases, and symptomatic cases are observed in this limited number of Paget's disease cases reported in Chinese individuals.