Adrenocortical carcinomas are rare lesion and R0 surgical resection remain their only chance for cure. They can be diagnosed because of their secretion but also on symptoms in relation with bulky tumours. Large lesion with caval thrombus can be surgically challenging, especially, as in the present case, when hepatic outflow obstruction leads to Budd Chiari syndrome. The present video shows a nephro-adrenalectomy with en-bloc right hepatectomy and caval thrombectomy for adrenocortical carcinoma in a patient with a Budd Chiari syndrome.