Clinical presentation, kidney biopsy findings, and clinical outcomes of immunoglobulin A nephropathy (IgAN) are highly variable. The objective of this study is to study the clinical presentation, histologic patterns, and outcomes of IgAN in the Pakistani population, as no significant data are available in international literature from this part of the world. A retrospective chart review was conducted of all patients with biopsy-proven IgAN between January 2007 and December 2017. Of a total of 977 renal biopsies, 50 patients had biopsy-proven IgAN (5.1%). The median age at the time of biopsy was 34 years (27-42); 92% of patients were between 18 and 40 years. Thirty-eight (76%) were male. Ninety-two percent of patients had significant proteinuria of >1 g/day, with 32% having nephrotic range proteinuria. The mean estimated glomerular filtration rate (eGFR) at presentation was 46.58 mL/min/1.73 m2. Seventy-eight percent of patients were hypertensive at the time of presentation and most of them had uncontrolled hypertension (HTN). The most common lesion on light microscopy was focal necrotizing glomerulonephritis (26%), followed by mesangial expansion with segmental/global glomerulosclerosis (22%). Crescents were seen in 38% of cases. Of 50 patients, a follow-up of at least six months was available for 32 patients. Most of the patients who had an eGFR of <30 mL/min at presentation progressed to kidney failure at six-month follow-up period. IgAN usually presents in young male adults in the age range of 18-40 years, with most patients having severe clinical presentation characterized by nephrotic-range proteinuria, HTN, renal insufficiency, and severe histological stages.