Maffucci syndrome consists of skeletal chondromas and associated soft tissue angiomas. This study reports the case of a woman whose disease is largely limited to the hands. It is of particular interest because plastic and reconstructive surgery improved the appearance of the hand, reduced the size of the tumors, and made the hand less prone to trauma. Amputation was not necessary. Angiography demonstrated the multiple arteriovenous malformations in the tumors of the hand while electron microscopy substantiated that the cartilage cells were normal chondrocytes with no abnormalities of the cartilage matrix. Genetic studies revealed a normal caryotype pattern.