〔Abstract〕 Objective To investigate the incidence and genotype distribution of glucose-6-phosphate dehydrogenase (G6PD) deficiency in neonates in Ningde city, Fujian province, in order to prevent the acute onset of G6PD deficiency. Methods A retrospective study was conducted to investigate neonates born in Ningde City from April 2016 to January 2017 who received plantar blood for G6PD screening. Suspicious children were recalled by telephone for reexamination and genotype testing, special observation of the results of She nationality newborn. Results In this study, a total of 43192 neonates were screened for plantar blood, among which 699 neonates were initially positive, with a detection rate of 1.62 % (699/43192). There were 646 males (1.50 %) and 53 females (0.12 %). 528 cases were recalled, with a recall rate of 75.54%. 496 cases were retested positive, and the coincidence rate of retested positive was 93.93%. A total of 280 neonates (56.35%) were screened for G6PD deficiency, and 239 neonates were detected for G6PD deficiency, the coincidence rate was 85.36%. There were 64 cases of She nationality children, accounting for 22.86% of the recalled number, 53 cases were positive, and the positive coincidence rate was 82.81%. She nationality positive children accounted for 18.92% in the reexamination population. In this study, 10 genotypes were detected: c.1376G > T, c.1388G > A, c.95A > G, c.871G > A, c.392G > T, c.1360C > T, c.1024C > T, c.1387G > T, c.487G > A, c.493A > G. Among them, c. 1376G > T mutation was the most common, accounting for 53.55%. The common genotypes of G6PD deficiency in She population were c.1376G > T, c.1388G > A, c.95A > G and c.1024C > T. Conclusion The incidence of G6PD deficiency in neonates in Ningde city was 1.62 %. Due to the genetic characteristics of G6PD deficiency, the incidence of male neonates was significantly higher than that of female neonates. The genotypes c.1376G > T, c.1388G > A and c.95A > G were most common in Ningde area. c.1376G > T, c.1388G > A and c.95A > G are also common in She population.