Human immunodeficiency virus-associated nephropathy (HIVAN) has rarely been reported in African children. In this single-center study, we analyzed ten children diagnosed with HIVAN from January 2000 to October 2006. There were eight boys and two girls, with a male:female ratio of 4:1. Their ages were from 5 months to 15 years (mean 6.8+/-6.2 years), with a peak age of 5-9 years. The presenting complaints included generalized edema (60%) and hypertension (50%). All patients had proteinuria on urine dipstick, with four (40%) at nephrotic range (proteinuria >or=500 mg/dl). Nine (90%) patients were in renal failure, with elevated serum creatinine (6.3-24 mg/dl) and serum urea (70-120 mg/dl). Renal disease was the first manifestation of HIV infection in six patients, whereas the diagnosis was made on autopsy in three. The duration from HIV infection to development of HIVAN ranged from 5 months to 10 years. CD4(+) cell count, done in only three patients due to financial constraints, was below 200/mm(3). The kidneys were hyperechoic on abdominal ultrasound in all patients, and three (30%) showed grossly enlarged kidneys. Histology of renal tissues available by autopsy in three patients showed mainly collapsing focal segmental glomerulosclerosis. Treatments given were angiotensin-converting enzyme (ACE) inhibitors and highly active antiretroviral therapy (HAART) in four and two patients, respectively, and one patient underwent peritoneal dialysis. On outcome analysis, seven (70%) patients died, two were lost to follow-up, and one was alive on HAART therapy at the writing of this article. In conclusion, HIVAN occurs in Nigeria children, and the mortality is very high from uremia.