Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. Treatment options for ACC are limited, with resection the main intervention. Most cases present in late metastatic cases, and data regarding effective therapies is limited. We report a case of ACC in a 40-year-old woman with history of ACC postadrenalectomy, who presented with recurrent metastatic ACC in the left perinephric space. She was started on pembrolizumab which was added to her mitotane maintenance therapy. Complete radiological response was achieved after 4 cycles of pembrolizumab. As far as we know, this is the first case to achieve complete radiological response with mitotane and pembrolizumab in recurrent metastatic ACC, with negative prognostic markers and no prior radiotherapy. As our findings are in the setting of one clinical case, we suggest the need to perform a trial to assess the benefit of combining mitotane and pembrolizumab in treating metastatic ACC.

肾上腺皮质癌（ACC）是一种罕见的恶性肿瘤，预后较差。ACC的治疗选择有限，以切除为主要干预手段。大多数病例出现在晚期转移病例中，有关有效疗法的数据有限。我们报道了一名40岁女性，患有ACC肾上腺切除术后的ACC病例，该患者在左肾周间隙出现复发转移性ACC。她开始使用pembrolizumab，该药物已加入米托坦维持疗法中。pembrolizumab的4个周期后实现了完全的放射学反应。据我们所知，这是第一个在复发转移性ACC中使用米诺坦和pembrolizumab达到完全放射学反应的病例，其预后指标为阴性，并且没有事先放疗。由于我们的发现是针对一个临床病例，