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.Primary leiomyosarcoma of the lung is a malignant tumour occurring rarely both in children [2-5, 6, 8, 9, 11] and adults [1, 2, 7, 10].The tumour arises from smooth muscle either of the bronchial or arterial walls and is characterised by a variable pattern of local growth and blood-borne metastatic spread, while lymphatics and lymph nodes are usually spared [2,7,10].