Objective To investigate the diagnosis and treatment of Chediak-Higashi syndrome(CHS) in accelerated phase. Methods Report a case and search the case reports of Chediak-Higashi syndrome in domestic literature, and collect the clinical datas for statistical analysis. Results 34 articles, a total of 52 cases were recruited. Male: female ratio of 1.17:1; Twenty five percent patients had family history; in 25.8% consanguineous marriage. 92.3% patients were hospitalized because of fever, many of them had repeated infection history, 97.9% had skin or hair pigmentation disorders; 78.8% had enlarged lymph nodes, 86.5% had splenomegaly and enlargement of liver. More than 64.2% patients had cytopenias (affected more than two lineages). The giant inclusions in the cytoplasmic of white cells in blood or bone marrow smear were found in all cases. The improvement rate in Chemotherapy group was higher than in Un-chemotherapy group (100% vs 50%, P=0.005). Invalid and death group compared with improvement group, age (years) (1.42 ± 1.30 vs 3.61 ± 1.81 P =0.0016), spleen (cm) (6.1 ± 3.4 vs 3.5 ± 2.1, P=0.02), liver (cm) (4.3 ± 2.4 vs 3.0 ± 1.4, P=0.097), cytopenias (affecting more than two of three lineages in the peripheral blood) (66.7% vs 55.6%, P=0.58). Conclusion Most of CHS patients showed recurrent fever, splenomegaly, enlargement of liver and lymph nodes, cytopenias (affected more than two lineages), which were not enough for diagnosis of a accelerated phase. The age of onset could be indication of chemotherapy, early onset of illness was associated with high mortality and chemotherapy helped to control the disease.