Primary Ewing's sarcoma/primitive neuroectodermal tumour (ES/PNET) of the kidney is a rare neoplasm characterized by an aggressive clinical course. Most often, ES/PNET arises in the chest wall and paraspinal locations, and less commonly in the limbs or the genitourinary tract. We present a rare case of renal ES/PNET presenting as Budd-Chiari syndrome.