Pachydermodactyly is a rare form of superficial digital fibromatosis characterized by progressive asymptomatic thickening of the back and sides of the proximal interphalangeal joints of the fingers. Atrophia maculosa varioliformis cutis is an acquired dermal atrophy, localized on the cheeks. Only a few cases of each pathology have been published. We find it interesting to report the case of a patient with both conditions as these two connective-tissue diseases are very rare. The association is probably fortuitous.