There are two types of congenital enlargement of the finger. In one type, the finger is enlarged as part of a syndrome or because of a tumor or a malformation. In the other, the finger is enlarged as an isolated anomaly called "true macrodactyly", with an abundance of fat mainly on the palmar side of the finger. This condition is a rare congenital malformation and the etiology is unknow. The enlarged portion of the finger or the hand can be in a distribution of a major peripheral nerve, most often the median nerve. This condition is called nerve territory-oriented macrodactyly. Macrodactyly can be static, present at birth and does not increase disproportionately with growth, or progressive with a disproportionate growth of the digit. Treatments are difficult and challenging. The goals of operative treatments are: to control the growth in children, mainly with epiphysiodesis; to reduce the size of the finger with resection of skin and subcutaneous tissue with occasionally recession osteotomy, angulation osteotomy and arthrodesis; amputation in severe progressive macrodactyly. Parents should be informed of the potentiel number of operations necessary and of the limitation of function.