The clinicopathological distinctiveness of nodular lymphocyte-predominant Hodgkin's lymphoma (NLPHL) is now unanimously recognized. Occasionally, NLPHL lesions are difficult to distinguish from T-cell/histiocyte-rich large B-cell lymphoma (THR-BCL), a morphological variant of diffuse large B-cell lymphoma. It has been suggested that THR-BCL represents a phenotypically peculiar expression or an evolutionary stage of NLPHL. Here, we present a review of both disorders, integrating our own experience with recent evidence from the literature. Overall, currently available data indicate that both diseases constitute intrinsically different entities that may, however, originate from a comparable transformed germinal centre ancestor.