Malignant change in existing benign enchondroma of phalanx of hand to chondrosarcoma of hand is extremely rare. Books suggest that chondrosarcoma does not arise in small bones of hands and feet although in literature few cases have been reported but not described comprehensively.

We report a rare case of chondrosarcoma in distal phalanx of ring finger in a 75- year old healthy female who had cystic lesion for past 25 years with recurrent fractures. Patient came to us with severe pain and tender, hard swelling of distal phalanx of left ring finger. X ray showed pronounced expansion of the terminal phalanx surrounded partially by a shell of bone, with focal spotty calcification with in the lesion. Because of sudden increase in size and pain of swelling, an amputation was performed at distal inter phalangeal joint. Histopathology showed grade II myxoid chondrosarcoma with pre-existing enchondroma. Wound healed nicely. Extensive investigation in form of CT chest and bone scan did not show any metastasis. Five year follow up did not show any local recurrence or distant metastasis.

Clinical suspicion should be aroused in an older individual with a previously relatively quiescent lesion that becomes larger and painful. Usually course of the tumour is slow and metastasis to lungs is late. Treatment of choice is disarticulation a joint proximal to lesion. Prognosis is good if metastasis has not occurred. Once diagnosis is made, patient should be investigated to look for any possible metastasis and must be regularly followed up. A literature review and discussion of salient diagnostic and treatment issues is included.