Background: Wilms’ tumor being the commonest primary malignant renal tumor in childhood, makes Surgeons rarely think of other aetiologies. Renal cell carcinoma (RCC) in paediatric age group is a rare entity and most children with renal tumor are diagnosed unexpectedly following nephrectomy for presumed Wilms’ tumor. Case presentation: We report an 8-year-old boy presenting with lump in the abdomen and haematuria for past 5 years. Abdominal ultrasound revealed a left renal mass measuring 10 × 10 cms, and a clinical diagnosis of Wilms’ tumor was made. Radical nephrectomy was performed, following which histological diagnosis of clear cell variant of renal cell carcinoma was made. Conclusion: The possibility of a paediatric renal cell carcinoma should be borne in mind when a patient presents with pain in the flank and a renal mass. RCC in a child is often a histological surprise and needs documentationinthe literature.