Adult polycystic kidney disease is transmitted in autosomal dominant pattern with a reported incidence of 1 in 1000 to 1 in 5000 cases. Horseshoe kidney is a renal fusion anomaly with an incidence of 1 in 400 to 1 in 1800 live births. Polycystic horseshoe kidney is a rare occurrence of 1 in 134,000 to 1 in 8,000,000. We here present a patient who was admitted with left-sided flank pain, hypertension and polycystic horseshoe kidney.