Objective <p>To evaluate the clinical significance of the ileocecal valve (ICV) in pediatric short bowel syndrome (SBS) regarding parenteral nutrition (PN) dependence, nutritional status, and complications.</p> Methods <p>A retrospective cohort study was conducted at Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University, involving 140 pediatric SBS patients. Patients were divided into ICV-preserved (<i>n</i> = 80) and ICV-absent (<i>n</i> = 60) groups. Outcomes assessed included PN weaning success, PN duration, intestinal failure-associated liver disease (IFALD), catheter-related bloodstream infections (CRBSI), deficiencies in vitamin D and trace elements, and liver function parameters.</p> Results <p>Compared with the ICV-absent group, ICV-preserved patients had a higher PN weaning success rate (88.0% vs. 71.2%, <i>p</i> = 0.015), a shorter median PN duration (107 vs. 160 days, <i>p</i> = 0.004), and a lower incidence of CRBSI (37.3% vs. 64.4%, <i>p</i> = 0.002). No significant difference was observed in IFALD incidence (42.7% vs. 57.6%, <i>p</i> = 0.086). Among ICV-absent patients, preservation of &gt; 50% of the colon was associated with improved PN weaning success (77.8% vs. 50.0%, <i>p</i> = 0.045). Initial stoma creation (vs. primary anastomosis) was associated with higher rates of IFALD (56.1% vs. 38.5%, <i>p</i> = 0.047) and CRBSI (57.3% vs. 36.5%, <i>p</i> = 0.019). Vitamin D deficiency was highly prevalent (86.5%) with no significant difference between groups.</p> Conclusion <p>ICV preservation in pediatric SBS is associated with reduced PN dependence and lower CRBSI risk, underscoring its clinical importance.</p>