Background Urological complications are the major disease manifestation in patients with spina bifida. Urinary sepsis accounted for the majority of admissions in these patients. As the patient grows older, changes occur in the bladder, leading to increases in storage pressure and consequent risk of deterioration of renal function, which may occur insidiously. Case presentation A 26-year-old male spinal bifida patient had been on ureterostomy for his neuropathic bladder since 2019. This patient was advised intermittent catheterisations. Following a routine change of urethral catheter, this patient became unwell and self-catheterisation was not possible because of long, overhanging prepuce and marked spinal curvature. This patient developed repeated urine infections. Five years later, the patient was admitted to the ICU because of severe headache, vomiting, no intake, severe stomach pain, unstable vital signs, no urine output, laboratory examination show ureum 354 mg/dl, creatinine 12,4 mg/dl (baseline 3), MRI examination of urinary tract revealed moderate hydronephrotic left kidney with hydroureter. There was no evidence of dilatation of the ureter near the vesicoureteric junction. The left kidney appeared normal. There was no evidence of stones in either kidneys. Ureterostomy drainage was then established. The right kidney showed under performed and morphology contracted. Under adequate treatment and good kidney function surveillance (KFS) based on guideline, patient recovered from severe urosepsis and was discharged a week after hospital admissions. Conclusion Although ureterostomy may be convenient for a spina bifida patient and the carers, hydronephrosis can occur insidiously. With recurrent urine infections, hydronephrotic kidney can become infected, which is life-threatening. Therefore, every effort should be made including KFS. Successful implementation of KFS in spinal cord injury patients requires education of the patient, availability of carers, who have been trained to perform ureterostomy cleaning and facilities for ureterostomy care wether in the house as well as in public places.