Preserving the ileocecal valve (ICV) has shown significant benefits. We present our experience with 18 infants who underwent ileocecal valve-preservation ileocecostomy (IVPI) with an extremely short distal ileum after primary ileostomy. A retrospective analysis was conducted on IVPI cases between 2014 and 2020. Medical records were reviewed, including birth weight, age, primary diseases, length of ileus stump, surgical time and procedure, time to enteral feeding, postoperative hospital stay, and complications. Eighteen patients (male: female = 12:6, median birth weight 1305 (750-4000) g, median gestational age 29 + 5 (27 + 6-39 + 6) weeks) were included in the analysis. Causes of surgery included necrotizing enterocolitis (13), ileocecal intestinal atresia (1), ileum volvulus (2), meconium peritonitis (1), and secondary intestinal fistula (1). The median corrected age of ileostomy closure was 3.2months (2.0-8.0months). The distance from the distal ileal stoma to the ICV ranged from 0.5 to 2cm. The median length of the residual bowel was 90cm (50-130cm). ICV-plasty was performed in 3 cases due to secondary ICV occlusion or stenosis. All patients resumed feeding within 6 to 11days after surgery. The postoperative hospital stay ranged from 12 to 108days (median: 16.5days). Complications included incisional infections in 2 cases, anastomotic stricture and adhesive ileus in 1 case, nosocomial sepsis and septic shock in 1 case. All children showed normal growth and development during a 6-65month follow-up. IVPI is safe and feasible for infants with an extremely short distal ileal stump. ICV-plasty could be applicable for cases with ileocecal occlusion/stenosis.