Primary malignant tumours of the pleura are classified into malignant mesotheliomas, mesenchymal tumours and primary lymphomas. The incidence of malignant pleural mesothelioma is increasing throughout most of the world and is an aggressive, treatment-resistant tumour. Its exact prevalence is unknown but it is estimated that represent less than 1% of all cancers. It has a highly specific causal relationship with asbestos and more than 80% of mesotheliomas are attributable to asbestos exposure. The latency period from asbestos exposure to development of the tumour is 20–40 years. Mesotheliomas with indeterminate (idiopathic) aetiology (without asbestos exposure) also occur but are very rare, less than 1 per million. Tumour usually presents in the fifth to seventh decade, mostly in men. At an early stage of tumour development small nodules are found in the parietal pleura that eventually extend along the whole pleural surface and tumour encloses the entire lung parenchyma. Patients typically present with breathlessness and chest pain with pleural effusions. Mesotheliomas can be categorized histologically as epitheloid, sarcomatoid, desmoplastic and biphasic type. Median survival time is about one year. Primary mesenchymal tumours of pleura and lymphomas are rare neoplasms most of them with aggressive behaviour and short survival period.