The authors report the case of a 2-year-old girl with pulmonary cystic adenomatoid malformation type II who presented with a mass on the lower lobe of the left lung. Resection and histological examination revealed pleomorphic rhabdomyosarcoma. Chemotherapy and radiotherapy followed the operation and the girl is alive and in perfect condition 10 years after the operation. The literature on primary rhabdomyosarcoma of the lung in children is reviewed.
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