Pleural effusion in young children is most commonly associated with infectious or inflammatory conditions. However, rare malignant causes should also be considered, particularly when symptoms persist despite standard interventions. A 2-year-old boy presented with cough, fever, and dyspnea unresponsive to initial pleural drainage. Imaging revealed a large, complex left pleural effusion with mediastinal shift. Surgical exploration uncovered a cystic lesion in the upper lobe of the left lung. Histopathological analysis confirmed pulmonary pleomorphic rhabdomyosarcoma – a rare and aggressive pediatric malignancy. The lesion was resected, and the patient’s condition improved postoperatively. A regional lymph node was tumor-free. The child was referred for further oncologic care. Although extremely rare, pulmonary pleomorphic rhabdomyosarcoma should be included in the differential diagnosis of persistent pleural effusion in children. Early surgical intervention and histological confirmation are essential for timely diagnosis and initiation of appropriate therapy
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