Background: Anorectal malformations (ARMs) are often associated with tethered cord syndrome (TCS). This study focused on children with ARM to explore the risk factors for the co-occurrence of TCS and to investigate the impact of untethering surgery on anorectal function among these children. Methods: A retrospective analysis was conducted on 130 children with ARM treated at Fujian Provincial Children's Hospital (Fujian Hospital of Shanghai Children's Medical Center) from May 2021 to January 2024. A total of 114 children were included in the study on the basis of the inclusion and exclusion criteria. The patients were divided into two groups according to the presence of TCS: the ARM group (n=83) and the ARM+TCS group (n=31). to explore the risk factors for the co-occurrence of ARM and TCS. All children diagnosed with TCS underwent untethering surgery regardless of symptoms. Anorectal function was compared between the ARM and ARM+TCS groups. Results: In the multivariate analysis, intermediate or high-type ARM increased the risk of children with ARM having TCS, with an OR of 3.571, 95% CI from 0.106 to 0.738, and p= 0.010. Additionally, the presence of other malformations also increased the risk of children with ARM having TCS (p=0.026). When the ARM+TCS group was compared with the ARM group, children with low-type ARM in the ARM+TCS group exhibited a significant improvement in constipation following untethering surgery (p=0.043). However, when children with intermediate or high-type ARM were compared, the anorectal function of the children in both groups was comparable. Conclusions: Intermediate or high-type ARM and the presence of other malformations are risk factors for the co-occurrence of TCS in children with ARM. In children with low-type ARM, those with TCS and ARM showed significant improvement in constipation after untethering surgery compared with those with ARM without TCS. We recommend that children with relevant conditions actively undergo untethering surgery.