Background: Hemophagocytic lymphohistiocytosis (HLH) a hyperinflammation disease and have high early mortality. The neutrophil-lymphocyte ratio (NLR) plays a prognostic role in various inflammation conditions. However, the role of NLR in HLH remains unknown.Results: 92 patients were collected from Sep 2014 to Dec 2019, the median age was 50 years (16-88) and 54 patients (58.6%) were male. 39 patients (42.3%) died during a 30-day follow-up. In addition, NLR was higher in nonsurvivor than survivor (P=0.005), higher NLR was correlated with older age (P=0.020), worse survival status (P=0.006), lower HB level (P=0.008), longer PT level (P=0.010), higher creatinine level (0.027) and higher IL-6 level (P=0.008)and in multivariate analysis, NLR (HR=2.508, 95%CI: 1.275-4.934), was indicated as an independent prognostic factor for early death. Conclusions: NLR was correlated with early death in HLH, suggest NLR could be used as a reference indicator for the monitoring and management of HLH.