Leiomyosarcoma of the lung is a type of tumour rarely found in childhood. It arises from smooth muscle either of bronchial or arterial walls, has a variable pattern of local growth, blood-borne metastatic spread with lymph nodes sparing and a clinical course characterized by fever, cough and worsening dyspnea. We report a case which occurred in a 14-year-old girl, reviewing all the other cases found in the literature.