Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is an indolent CD20(+) lymphoma. Its scarcity has made clinical trials difficult and there is no consensus on first-line treatment. We conducted a population-based study of all patients diagnosed with NLPHL in Sweden between 2000 and 2014 (N=158; 41 women and 117 men), focusing on clinical features, therapy and overall survival. The median female and male age was 59 and 44years, respectively (P=0002). In early-stage disease, there was little mortality and no survival differences between therapies. In patients with advanced-stage disease, mortality was relatively high in patients who did not receive first-line rituximab but absent in those who did (10-year survival, 55% vs. 100%; P=0033); there were no imbalances of prognostic factors between those two groups. In advanced stages, first-line rituximab use increased markedly between 2000-2004 and 2005-2014 (7% vs. 67%; P<000005), as did 10-year survival, 53% vs. 72% (multivariate P=0027). Although all patients were diagnosed in the 2000s, this is the longest-followed (and largest) population-based cohort. We report a hitherto unreported 15-year median age difference between sexes, increasing rituximab use and improved survival.