BACKGROUND: Low- to intermediate-grade chondrosarcoma is usually a slow-growing and highly chemotherapyresistant tumor type. CASE REPORT: The 76-year-old female patient presented with low- to intermediate-grade chondrosarcoma of the distal phalanx of the right thumb in 1993 and was treated with an excision of the carpometacarpal joint. Approximately 10 years later, the patient presented with recurrent local disease at the trapezoid bone, which was resected. Eight months later she presented with local recurrence at the carpal joint and lung metastases. Subsequently she was treated chemotherapeutically with pegylated liposomal doxorubicin as part of a clinical trial. Due to tumor progression after 2 cycles, she was switched to oral trofosfamide (150 mg daily). A follow-up CT scan after 8 months demonstrated a partial remission that was confirmed by a second scan after 9 months of treatment. The treatment was well tolerated. After 18 months of continued treatment, she was still in remission and on trofosfamide. CONCLUSION: Trofosfamide at a daily dose of 150 mg may be effective in low- to intermediate-grade chondrosarcoma.