Adrenocortical carcinoma (ACC) is unusual malignancy and occurs more frequently in women. It generally occurs sporadically, however it may occur as a part of Li–Fraumeni syndrome and Beckwith–Wiedemann syndrome. Imaging studies (CT scan, MRI and fluorodeoxyglucose-positron emission tomography) are used for diagnostic purposes. Tumor staging is performed by tumor-node-metastasis classification as suggested by European Network for the Study of Adrenal Tumors (ENSAT). Some hormonal investigations are also useful in the detection of recurrence. Fine-needle aspiration biopsy is useful in cases of suspected adrenal metastasis from another primary carcinoma. Surgery, adjuvant therapy, chemotherapy, radiotherapy and Emerging therapies for ACC (vascular endothelial growth factor receptor inhibitors, IGF-I receptor inhibitors, platelet-derived growth factor inhibitors, differentiation agents, and proteasome inhibitors) are used for the treatment of ACC.