Horseshoe kidney (HSK) is a common renal malformation with unique and complex characteristics. A systematic literature search was conducted using PubMed and ScienceDirect databases. Several theories have been proposed regarding HSK formation, such as the close apposition of the kidneys during ascent through an arterial fork, lateral flexion of the trunk, and caudal embryonic rotation. Emerging evidence from animal models implicates notochord signaling and the sonic hedgehog pathway in HSK formation. The isthmus, a defining feature of HSK, is hypothesized to arise from ectopic mesenchymal tissue. The surgical anatomy of HSK is complex, given the variability in location, orientation, and blood supply. Both arterial and venous anatomy exhibit significant variability, raising questions about whether anomalous blood supply is a cause or a consequence of abnormal renal position. The isthmus usually contains functional renal parenchyma and fusion between the kidneys, primarily at the lower pole. While it is often stated that the inferior mesenteric artery is "held back" at the L3 level, this anatomical configuration is present in only 40% of cases. The review highlights the need for further research and provides a comprehensive overview of HSK knowledge.