Chromophobe renal cell carcinoma (RCC), a subtype of RCC, accounts for 4-6% of all RCC and has better prognosis then conventional RCC. Sarcomatoid dedifferentiation is thought to represent the high-grade end of all subtypes. This makes chromophobe RCC with sarcomatoid changes a rare entity associated with poor prognosis in most studies. We present a case of a 40-year old female with this rare histology, with the tumour localised to the renal capsule, managed with nephrectomy and with close follow-up thereafter. The patient is free of disease after one year of treatment.