Adrenocortical sarcomatoid carcinoma (ASC) is an extremely rare variant of adrenocortical carcinoma (ACC). Its relative rarity and its characteristic histological pattern of both epithelioid and sarcomatoid components may pose diagnostic challenges which influence treatment. Here, we report a case of ASC in a 58 year-old man presenting with increasing abdominal pain and associated abdominal bloating with a large right adrenal mass detected by computed tomographic scan (CT). To our knowledge, only eleven prior cases of ASC have been reported in the literature. Here, we discuss the clinical, radiological and histopathological findings in our case, review the literature on ASCs and offer opinion on best management.