Renal oncocytosis in a pediatric patient: Case report and review of the literature

•Renal oncocytosis is a rare condition.•12 yr old female presented with flank pain and several masses in her right kidney.•Final pathology revealed multiple benign oncocytomas.•A review of the literature of this rare condition was performed.

Renal oncocytosis is a rare condition in which the kidney develops numerous oncocytomas. We present a case of a 12-year-old female who presented with right-sided flank pain of one-year duration. Imaging revealed several masses in the right kidney. Tissue biopsy confirmed multiple benign oncocytomas. Due to the presence of multiple oncocytomas throughout the kidney, a radical nephrectomy was performed. Given the rarity of this condition, as well as its known association with von Hippel-Lindau disease and Birt-Hogg-Dube syndrome, genetic investigations were pursued but failed to identify any abnormalities. This patient remains well and disease free six years after surgery. A review of the literature of this rare condition was performed.