Immunoglobulin A-dominant acute postinfectious glomerulonephritis (IgA-dominant APIGN) is an increasingly recognized morphologic variant of APIGN. In contrast to classic APIGN, in which there is glomerular deposition of IgG and/or C3, IgA is the sole or dominant immunoglobulin deposited in IgA-dominant APIGN. Patients present with declining glomerular filtration rate (GFR), proteinuria, and microscopic hematuria. Horseshoe kidney (HSK) is a relatively common renal anomaly. To this point, the co-occurrence of HSK and IgA-dominant APIGN has not been reported in the literature. Here, we report a case of IgA-dominant APIGN in a patient with HSK resulting in the need for temporary hemodialysis.