Spontaneous rupture of the ureter is a very rare condition and usually results from ureteral obstruction by a calculus. Only theoretical mechanisms have been proposed and no possible explanation has yet been reported in the literature. Intravenous contrast-enhanced computed tomography is the most informative study with high sensitivity. Treatment should be individualised, and depends on the state of the patient. Minimally invasive endourological procedures with double-J catheter placement and percutaneous drainage offer excellent results. Conservative management with analgesics and antibiotic coverage may be an alternative to surgery. Herein, we present a case of spontaneous rupture of the proximal ureter with no evidence of an underlying pathological condition.