Horseshoe kidney is a renal fusion anomaly during embryogenesis and adult polycystic kidney disease is a hereditary disorder which is transmitted in autosomal dominant pattern. Polycystic and horseshoe kidney are two separate disease entities, only about 20 cases of polycystic kidney disease in horseshoe kidney disease have been described in the literature, with an incidence ranging from 1 in 134 000 to 1 in 8 000 000 live births. We are presenting ultrasound findings of a patient who was incidentally diagnosed with polycystic horseshoe kidney on routine screening.