Abstract：Objective: To discuss the clinical application value of Ion AmpliSeq^TM Inherited Disease Panel semiconductor chip sequencing in genetic diagnosis for metabolic diseases. Methods: Peripheral blood samples of 109 children patients and their parents, who were suspected as inborn errors of metabolism diseases by neonatal screening with tandem massspectrometry, were collected. Ion AmpliSeq^TM Inherited Disease Panel was carried out and the suspicious mutations were verified by Sanger sequencing. Results: In the 109 cases, 97 cases (88.99%) allowed definitely genetic diagnosis. Among them, pathogenic mutations of two alleles were detected in 96 cases. A case of ornithine transcarbamylase deficiency was male hemizygous. Only one pathogenic mutation was detected in 11 cases (10.09 %). No pathogenic mutation was detectable in the 1 case (0.92%). Conclusion: Ion AmpliSeq^TM Inherited Disease Panel may exhibit high positive rate in the application for the diagnosis of the children with high risk of inborn errors of metabolism diseases and provide the evidences for the subsequent clinical treatment and genetic counseling.