Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare subtype of Hodgkin lymphoma that differs from classic Hodgkin lymphoma (cHL) with respect to histologic and clinical presentation. Because the prognosis of NLPHL in early unfavorable and advanced stages is similar to that of cHL, treatment is similar. In contrast, early favorable-stage NLPHL has a better prognosis than cHL. Thus, NLPHL in early favorable stages might be treated with reduced-intensity programs without compromising cure rates. Because involved-field radiotherapy alone seems to be as effective as extended-field radiotherapy or combined modalities, it has been adopted by the German Hodgkin Study Group and the European Organisation for Research and Treatment of Cancer as the treatment of choice for stage IA NLPHL. Now that efficacy of the monoclonal antibody rituximab has been shown in relapsed NLPHL, its use in the first-line treatment of NLPHL is under investigation.