Lymphocyte predominance Hodgkin's disease (LPHD) can be histologically subdivided into a nodular and diffuse variety. The two subtypes differ in immunophenotypic characteristics but have a similar long-term clinical outcome. Nodular LPHD has immunophenotypic and histological characteristics suggestive of a B-cell derived neoplastic process. Nodular LPHD is associated with an increased risk of secondary large cell lymphoma (LCL). Gene rearrangement studies in some of these cases have revealed a B-cell clonal process, further supporting the association between nodular LPHD and the B-cell system. In addition, it has been suggested that the apparent secondary LCL, at least in some cases, may represent a histologic progression of nodular LPHD. We report a unique case of T-cell lymphoma, confirmed by T-cell receptor gene rearrangement studies, which developed in the setting of nodular LPHD. Our observation demonstrates that the association of nodular LPHD and LCL is complex and that LCL developing in the context of nodular LPHD may be an independent secondary process sometimes involving T-cell lymphomas.