Incidentally detected renal cell carcinoma(RCC) on renal biopsy performed for reasons other than for investigating renal mass is rare. The incidence and natural history of this entity remain uncertain. We report a case of a patient with an incidentally discovered RCC on renal biopsy performed to evaluate nephrotic syndrome. The patient was followed up regularly and eventually underwent radical nephrectomy 30 months later, which confirmed clear cell RCC. Initial surveillance may be a reasonable option for patients with incidental RCC discovered on renal biopsy until the lesion is detectable on imaging study.