Tuberous sclerosis complex (TSC) is an extremely variable genetic disorder that can affect virtually any organ in the body. Disease manifestations continue to develop over the lifetime of an affected individual. Many manifestations can be life threatening; appropriate surveillance and management are necessary to limit morbidity and mortality in this disease. We report a case of an 8-year-old girl with TSC and bilateral renal cell carcinoma, which is usually thought to be a complication diagnosed in adulthood. Our report emphasises the need for frequent surveillance and renal imaging in paediatric patients with TSC.