Spontaneous steinstrasse, defined as an accumulation of ureteric stones without a preceding intervention such as extracorporeal shock wave lithotripsy (ESWL) or endoscopic lithotripsy, is an uncommon clinical entity. While post-procedural steinstrasse is well-documented, spontaneous cases remain rare and poorly understood. This case highlights an unusual presentation of spontaneous steinstrasse in a patient with recurrent nephrolithiasis and a large stone burden, requiring multiple endoscopic procedures. The presence of uric acid stones further complicated management, necessitating a combination of surgical and medical therapy.

A 40-year-old male with a history of recurrent nephrolithiasis, type 2 diabetes mellitus, and hypertension presented to the emergency department with acute left flank pain. CT KUB revealed ten small stones in the left ureter (steinstrasse), a 1.3 cm stone in the left renal pelvis, and multiple bilateral renal calculi. In the absence of infection, initial management included analgesia and tamsulosin.

Due to persistent symptoms, the patient underwent cystoscopy with JJ stent placement, followed by ureteroscopy with laser lithotripsy one month later, which cleared the ureteric stones but left residual renal calculi. A subsequent ureteroscopy further reduced the stone burden, with a final procedure achieving complete clearance. During treatment, a right distal ureteric stone passed spontaneously. Stone analysis confirmed 100% uric acid composition, leading to initiation of allopurinol and urinary alkalinization. Follow-up CT KUB showed no residual ureteric stones and a significant reduction in renal stone burden. The patient was discharged with ongoing medical management to prevent recurrence.

This case underscores the importance of recognising spontaneous steinstrasse as a distinct clinical entity requiring a tailored management approach. While ureteroscopy with laser lithotripsy remains the cornerstone of treatment for symptomatic cases, adjunctive medical therapies such as urinary alkalinization are crucial for uric acid stone prevention. This case highlights the need for vigilance in patients with metabolic stone disease and large stone burdens, as they may be at risk for spontaneous ureteric obstruction. Future studies should explore the metabolic factors contributing to spontaneous steinstrasse and the role of early medical intervention in reducing the need for multiple endoscopic procedures.