肾上腺皮质癌(adrenocortical carcinoma,ACC)罕见,临床表现多样,肿瘤进展快,预后差。CT为首选检查方法,MRI有其优点,检测相关激素有助于诊断。根治性手术切除是可能根治ACC的唯一方法。现能成功地行腹腔镜下肾上腺切除术治疗ACC,微创手术将来能否成为治疗ACC的优先方法,有待于 Adrenocortical carcinoma (ACC) is rare, with diverse clinical manifestations, rapid tumor progression, and poor prognosis. CT is the preferred method of examination, and MRI has its advantages. Detection of related hormones is helpful for diagnosis. Radical surgical resection is the only way to cure ACC. Laparoscopic adrenalectomy can successfully be performed for the treatment of ACC. Whether minimally invasive surgery will become the preferred method of treating ACC in the future remains to be addressed.