ABSTRACT: The management of upper urinary tract stones in patients with spina bifida is challenging but poorly described in the literature. We compared urolithiasis interventions and related complications in patients with spina bifida to those in other stone formers using a national database.We retrospectively reviewed the NIS to identify hospital admissions for renal and ureteral stones from 1998 to 2011. We used ICD-9-CM codes to identify urological interventions, including shock wave lithotripsy, ureteroscopy, percutaneous nephrolithotomy and ureteral stent placement. NSQIP data were used to identify postoperative complications.We identified 4,287,529 weighted stone hospital admissions, including 12,315 (0.3%) of patients with spina bifida. Compared to those without spina bifida the patients with spina bifida who had urolithiasis were significantly younger (mean age 34 vs 53 years), more likely to have public insurance (72% vs 44%) and renal vs ureteral calculi (81% vs 58%), and undergo percutaneous nephrolithotomy (27% vs 8%). After adjusting for age, insurance, comorbidity, treatment year, surgery type, stone location and hospital factors patients with spina bifida were more likely to have urinary tract infections (OR 2.5), urinary complications (OR 3.1), acute renal failure (OR 1.9), respiratory complications (OR 2.0), pneumonia (OR 1.5), respiratory insufficiency (OR 3.2), prolonged mechanical ventilation (OR 3.2), sepsis (OR 2.7), pulmonary embolism (OR 3.0), cardiac complications (OR 2.4) and bleeding (OR 1.6).Compared to those without spina bifida the patients with spina bifida who were hospitalized for urolithiasis were younger, and more likely to have renal stones and undergo percutaneous nephrolithotomy. Urolithiasis procedures in patients with spina bifida were associated with a significantly higher risk of in-hospital postoperative complications.