Most patients with pelvic kidneys are asymptomatic, and the abnormality is either incidentally noted or never discovered. A subset of individuals, however, may become symptomatic secondary to the development of an upper urinary tract obstruction, nephrolithiasis, or urinary tract infection. These sequelae occur if malrotation results in high insertion of the ureter or a vessel crossing the collecting system, since these can both cause urinary stasis and outflow obstruction.

Thus, patients with pelvic kidneys may occasionally have abdominal pain, hematuria, or a palpable abdominal mass. The pelvic kidney is then detected on further workup with ultrasound or computed tomography (CT). The treatment strategies for nephrolithiasis and ureteropelvic junction obstructions in patients with pelvic kidneys are largely the same as those used for patients with normally positioned kidneys; however, the abnormal course of the ureter may make ureteroscopy difficult, and there is a risk of damaging abnormally positioned vessels and nerves.