To determine the patterns of renal disease among Jordanian children based on kidney biopsy results as well as correlating clinical presentation with histopathological diagnosis, we studied the medical records of 55 children who had kidney-biopsy kidney disease at the Prince Hamza Hospital from August 2006 to April 2012. The most common indication for biopsies was steroid-resistant nephrotic syndrome (25%), followed by steroid-dependent nephrotic syndrome (20%). The most common diagnosis of primary glomerular disease was minimal change disease (27%), followed by focal segmental glomerulosclerosis (21%), mesangioproliferative glomerulonephritis (12.7%) and IgA nephropathy (7.3%). The most common secondary renal disease was Henoch-Schonlein purpura (HSP) in 10.9% of the patients, followed by systemic lupus erythematosus in 9.1%. The kidney biopsy was normal in 3.6%. Cyclosporine protocol biopsy was performed in four (7.3%) patients. Gross hematuria after kidney biopsy occurred in 5.5% of cases; however, none of these patients required blood transfusion. Kidney biopsy is a safe procedure that needs to be performed once indicated and our results are comparable with those carried out elsewhere in Jordan. The study also shows the importance of establishing a national registry for pediatric renal disease.