Horseshoe kidney (HSK) is the most common kidney malformation. This review analyses the literature surrounding the etiology, morphology and clinical anatomy of these kidney fusion anomalies. A systematic literature search was carried out using the Science Direct and Scopus applications. HSK is characterized by three anatomic abnormalities: ectopia, malrotation and vascular changes. The study put emphasis especially on blood supply and upper urinary tract changes in HSK's patients. Arterial blood supply was analysed not only basing on Graves pattern, but also a new model of supply created on horseshoe kidneys computed tomography was taken into account. In addition, study presents clinical aspects of especially significant pathology of HSK, like hydronephrosis, stones and urinary tract infection. This study focuses also on congenital anomalies associated with horseshoe kidney.