An 18-year-old male patient with a history of cystinosis and inflammatory bowel disease, who was being treated with cysteamine and 6-mercaptopurine, had an incidental finding of a mass in the right kidney on a renal ultrasound to evaluate worsening renal insufficiency. Excisional biopsy demonstrated papillary renal cell carcinoma, Type 2. Renal cell carcinoma is typically a disease of older adults, and it is not known to be associated with cystinosis. This is the first published report of renal cell carcinoma in a patient with cystinosis.