Pachydermodactyly is a kind of benign fibromatosis in which asymptomatic symmetrical soft tissue swellings occur on the proximal phalanges and the proximal interphalangeal joints of the hands. Although young women can also be affected, the young men are affected most commonly. There is no bony or articular abnormality on radiographic study. Histological examination of skin demonstrated epidermal hyperplasia, hyperkeratosis, acanthosis and thickened reticular dermis by deposition of collagen. It is important to recognize this disease identity without misdiagnosis for other rheumatologic diseases to avoid inappropriate and possibly toxic treatments. We report a case of pachydermodactyly and discuss the differential diagnosis.