OBJECTIVE Vesicoureteral reflux in children with myelodysplasia is usually secondary to abnormal bladder storage. The purpose of this study was to assess the outcome of vesicoureteral reflux in children with myelodysplasia. METHODS We retrospectively analyzed the records of 319 children with myelodysplasia who presented to our institution between 1978 and 1985. Of these children 95 presented with or had reflux during followup and they were treated with prophylactic antibiotics. Clean intermittent catheterization and anticholinergic medication were added to the regimen when indicated. RESULTS Reflux resolved in 63% of these patients with nonsurgical management. Temporary cutaneous vesicostomy was performed in 23 children (24%) with persistent high grade reflux or evidence of upper tract deterioration. Ureteral reimplantation and augmentation cystoplasty were performed in 18 (20%) and 8 (8%) patients, respectively. No patient had progression to chronic renal failure and scars developed in only 14 kidneys (10%). CONCLUSIONS In the majority of cases (63%) reflux resolved with nonsurgical management. Reflux in these patients should not be treated in isolation. The management of reflux is primarily aimed at improving bladder storage. The combination of aggressive nonsurgical therapy and close observation is recommended. This regimen leads to the satisfactory resolution of reflux with minimal renal morbidity.