Adrenal cortical carcinoma (ACC) is a rare aggressive tumor, with an annual incidence of 0.05-0.2/100.000 persons and a bimodal age distribution, with two peaks, in the first and fifth decade of life. The 4th WHO Classification of Tumours of Endocrine organs edited in 2017 recognizes four hystological variants of adult ACC: conventional, oncocytic, myxoid and sarcomatoid, in decreasing order of frequency.