One rare form of lung cancer that appears to be unique among people exposed to asbestos is known as mesothelioma. This cancer affects the lining of the lungs or abdomen. The association of asbestos exposure and malignant mesothleioma has been well documented. Approximately 80% of mesothelioma patients have along history of asbestos exposure. The combined roles of genetic and environment in the pathogenesis of mesothelioma has also been suggested by its familial aggregation. Studies show an association between single nucleotide polymorphisms leading to a defect in DNA repair poses an increased risk of mesothelioma. It is essential to identify genetic risk factors for malignant mesothelioma. The toxic asbestos fibers chronically retained in the lung can be carcinogenic as a result of: mechanical effects, such as interference during chromosome segregation; generation of reactive oxygen species; and local and systemic immunosuppression. Identification of specific risk genotypes will provide better estimation of population disease risk resulting from defined exposure; improve knowledge on lung carcinogenesis; and help target early detection of disease in high-risk individuals. It is a malignant neoplasm that may not appear for as many as 20 to 40 years following the exposure of the substance. This type of malignancy has a high fatality rate. The relation between asbestos exposure and mesothelioma is so close that the occurrence of this malignancy may be grounds for occupational compensation.