A Rare Case of Primitive Neuroectodermal Tumour (Pnet) of Kidney with Review of Literature

We report a case of primitive neuroectodermal tumour of the kidney in a middle aged male who presented as a large left sided renal mass similar to renal cell carcinoma. A cytoreductive nephrectome was done which showed primitive neuroectodermal tumour in the left kidney. A Positron Emission Tomography CT scan was done which showed metastasis in the lung and para-aortic lymph nodes. We describe the natural history, clinical and pathological features and management strategy of this rare variety of tumour.