Collagenomas or connective tissue nevi of the collagen type are hamartomatous growths of otherwise normal collagen. They can be hereditary or sporadic. We report an interesting case of isolated collagenoma confined to the right palm, without associated abnormalities, in a 6-year-old boy. To our knowledge, only one case of isolated palmar collagenoma has been reported in the English literature and this has not been described previously in the Korean literature.