The case of a 22-month-old boy with alveolar rhabdomyosarcoma of the lung is presented. Brain metastasis and recurrence of the right pulmonary hilum and parietal pleura developed 6, 11, and 24 months (respectively) after tumor resection. Chemotherapy and radiotherapy were effective. Neuron-specific enolase was very helpful in detecting metastasis and disease recurrence. Primary pulmonary rhabdomyosarcoma can be divided into two groups: tumor in the normal lung, and tumor in cystic lesions of the lung.