Adrenocortical carcinoma is a rare malignant tumor with poor prognosis and is highly aggressive.It is clinically more common in female patients.The cause of this disease has not yet been fully elucidated,but it may be related to organ dysfunction,dietary factors,and drug factors.It often manifests as virilization in females and hyperfunction of the adrenal glands.Due to the strong invasiveness of adrenocortical carcinoma and its propensity for metastasis,common lym-phatic metastases mainly involve the lymph nodes around the adrenal glands and the large arteries.Therefore,early detec-tion and complete surgical resection are key to treating the disease,with the aim of removing as much of the tumor and ad-jacent tissue as possible to increase the success rate of treatment.For patients with adrenocortical carcinoma who have dis-tant metastases or poor general condition and are not candidates for surgery,adjunctive therapies such as radiotherapy,chemotherapy,and molecular targeted therapy may be considered to prolong survival.This article presents a comprehensive and systematic review of the latest basic and clinical research advances in the treatment of adrenocortical carcinoma.The aim is to provide new perspectives and experiences in the clinical treatment of adrenocortical carcinoma.