Ureteropelvic junction (UPJ) obstruction is a well-recognized entity that may present at any time - in fetal life, infancy, childhood, or early or late adulthood. As the most common site of obstruction in the upper urinary tract, the UPJ is an area with which urologists should be well familiar. There has been an improved understanding of the pathophysiology of primary congenital UPJ obstruction that has been reflected in the evolution of surgical options, from open surgical repair to minimally invasive surgery. Although the primary scope of this review is the surgical management of this condition, we will briefly review the pathogenesis, clinical presentation, and diagnosis of UPJ obstruction.