| Summary: | Rhabdomyosarcoma is a rare malignant tumour occurring in the hand. Though the prognosis for this tumour is poor, when it occurs in the hand, early diagnosis is possible, since the swelling becomes clinically obvious. Radical surgical excision even at the cost of significant functional loss is recommended with possible reconstruction once the disease is under control. Advances in chemotherapy protocols and radiotherapy in selected cases has improved the prognosis. Rhabdomyosarcoma arising in the thumb of a four-year-old child is presented. He was treated by amputation of the thumb at the carpometacarpal joint level followed by chemotherapy. When he was disease-free at 18 months, thumb was reconstructed by pollicisation of the index finger. The child has remained recurrence-free at 30 months from diagnosis. The patient details are presented with review of the relevant literature.
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