Adrenocortical carcinoma is a very rare and aggressive endocrine tumor, which generally conveys poor overall 5-year survival. Worldwide incidence is estimated at 1-2 million /year. Patients who suffer from ACC can present with symptoms of hormone excess, including Cushing’s syndrome, virilization, feminization and less frequently hypertension associated with hypokalemia. In almost all cases, histopathological research can distinguish between adrenocortical adenoma and carcinoma, using one of the international classification methods. Other diagnostic techniques include hormone work up and imaging methods. Radical surgery is considered the therapy of choice in the first stages of ACC, and when surgery is not feasible, after incomplete resection or for metastatic disease, adjuvant mitotane is added. Control of excessive hormone production is a vital part of management in all patients. Nevertheless, despite complete resection, there are high recurrence rates. Management of recurrent ACC should take place in a specialist center and the individual biology of each case of recurrence must be considered when making treatment decisions. Early detection of recurrent disease is important as well as close follow up with clinical examination and imaging. Liver, pulmonary and bone metastasis are most often involved in systemic recurrence and management usually consists of mitotane, with or without combination chemotherapy. Surgical resection only plays a limited role in systemic recurrence, and only in selected patients. Unfortunately, treatment for this aggressive tumor is still ineffective.