A case of Maffucci's syndrome in a thirty-five year old woman who elected to have an above-the-knee amputation because of her disease is reported. The finding of a normal karyotype in this syndrome is reported, confirming previous observations. A brief review of the current literature reveals that seventy-five cases of Maffucci's syndrome have now been reported and that in fourteen of these sarcomatous degeneration occurred, giving a malignancy rate of 18.6 per cent.