Chondrosarcoma is the most common primary malignant bone tumour of the hand. This aggressive lesion is extremely rare in the distal phalanx and requires differential diagnosis with enchondroma and onycomatrichoma which are specific neoplasms of this phalanx. We report a case of chondrosarcoma arising from left index distal phalanx, which developed over a long period of time with a progressive deformity of the distal phalanx before becoming painful. The patient was investigated with X-ray and MRI scan which demonstrated a locally aggressive lesion. The surgical procedure was discussed with the patient and consisted of a wide resection with distal phalanx amputation; this surgery left an acceptable functional impairment. Recently, the optimal treatment for chondrosarcoma of the finger has been debated in the literature and a more conservative surgical approach has been proposed. Local recurrence and metastasis have been reported in the literature. Thus, after surgical treatment, the patients should be followed with careful postoperative surveillance.
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