Background <p>Adrenocortical carcinomas (ACC) are rare tumors of the adrenal gland. About onethird of patients present with metastatic disease at the time of diagnosis. Surgical removal of the tumor should only be performed when complete resection can be achieved. However, in selected cases, patients may profit from debulking surgery even if complete resection is not possible. Chemotherapy with EDP‑M (etoposide, doxorubicin, cisplatin, mitotane) is considered the standard of care.</p> Case report <p>A&#xa0;61-year-old female presented with hypertension, peripheral edema, and hirsutism. Diagnostic workup revealed metastatic adrenocortical carcinoma. Debulking surgery was performed. Due to post-surgical complications, no chemotherapy or further treatment—although initially planned—was given, and the patient passed away after 43&#xa0;days on best supportive care.</p> Conclusion <p>New therapeutic options in addition to conventional chemotherapy or radiation are required for metastatic ACC. Other treatment options include cytoreductive surgery with hyperthermic intraperitoneal chemotherapy or targeted therapies with immune checkpoint inhibitors, thus necessitating genetic testing. However, additional studies with larger sample sizes are needed to further evaluate these treatment strategies in this tumor entity.</p>