The horseshoe kidney (HSK) is the most common congenital abnormality of the upper urinary tract with an incidence of approximately 1 in 500 in the general population. Although individuals with HSK are often asymptomatic, they are at increased risk for neoplasms, infections, ureteropelvic obstruction secondary to lithiasis or vascular compression. Direct injury from trauma is increased in these individuals as is the risk of intraoperative complications secondary to damage involving the typically complex renal or adrenal vascular supply.We briefly review etiological factors including renal and urinary system embryology, genetic mutations, abnormalities related to faulty cell signaling, aberrant cell migration, and other possible causes including environmental exposures and trauma. In addition, we call attention to factors that might influence the success of surgical procedures in patients with HSK. We argue that an understanding of possible etiologies of the HSK and its different subtypes may be useful when planning surgical procedures or considering risk–benefit ratios associated with different surgical options.We briefly present the organization of a HSK in a 100‐year‐old male demonstrating an unusual vascular supply discovered during a dissection laboratory session in a medical school anatomy course. We describe the structure of the HSK, the position and relationships of the HSK to other structures within the abdomen, and the associated vascular relationships.