ObjectiveTo study the clinicopathological features of mediastinum nodular sclerosis Hodgkin lymphoma (NSHL) in order to improve the recognition of it. MethodsThe clinical data of 3 cases of mediastinum NSHL between 2003 and 2012 were collected. Then we analyzed the carcinoma pathologic samples by pathomorphology, immunophenotypic phenotype, related gene rearrangement and situ hybridization with EBER. ResultsThe pathomorphologic results showed that broad fibrotic bands subdivided the lymphoid parenchyma into large nodules, the tumoral cells had distinct boundary with empty cytoplasm and small-to-medium-sized nucleoli, and the nodules contained inflammatory cell components. The immunophenotypic phenotype of the tumoral cells were CD15, CD30, PAX-5 and CD20 partly, but anaplastic lymphoma kinase, CD45, cytokeratin, CD79α and S-100 were not expressed. T cell receptor γ and IgH gene were no rearranged, and EBER in situ hybridization was not detected. ConclusionVarious lymphomas occur in the mediastinum and mediastinum NSHL is just one of them. Mastering its distinctive pathomorphology and immunophenotypic phenotype is highly significant for diagnosis, differential diagnosis and treatment of the disease.