Nuclear gene-related mitochondrial complex deficiency is a group of inherited diseases characterized by oxidative phosphorylation dysfunction of mitochondrial respiratory chain caused by mutations in nuclear genes， and it is mainly inherited in an autosomal recessive pattern. The patients with this disease have the main clinical manifestations of fluency disorder and abnormal gait， accompanied by the specific clinical presentations of corresponding mitochondrial complex dysfunction， showing a certain degree of clinical heterogeneity. This article discusses the issues in screening carriers for nuclear gene-related mitochondrial complex deficiency and elaborates on this disease from the aspects such as screening methods， target populations， screening processes， and pre- and post-screening counseling， in order to standardize its application and provide better service for clinical practice. ［Journal of International Neurology and Neurosurgery, 2024, 51(5): 68-74］