Horseshoe kidney is a relatively common renal anomaly with which many structural and developmental anomalies have been shown to be associated. However, there are only a few case reports regarding the association of membranous glomerulonephritis and focal sclerosing glomerulonephritis in patients with horseshoe kidneys. We report a girl who was evaluated for hematuria and proteinuria, and found to have horseshoe kidney. Renal biopsy demonstrated mesangioproliferative glomerulonephritis. She also had simple diffuse goiter.