Background: Adrenocortical carcinoma (ACC) is a rare (1,02 per million population) disease with unfavorable prognosis. In Brazil, the incidence is about 15 times higher than in the rest of the world, mainly due to significant prevalence of the TP53-R337H germline mutation in this population. Methods: We performed a retrospective analysis of 66 patients diagnosed with ACC at AC Camargo Cancer Center. Clinical, pathological, epidemiological, treatment and survival data were collected from the patient’s chart and described. Results: Between 2004 and 2021, 66 patients were diagnosed with ACC. The median age was 45 years and 56% were female. 23 (35%) patients had at least one first-degree relative with a history of cancer and 8 (12%) had a second primary tumor. Ten patients were tested for Li-Fraumeni Syndrome and 30% went positive (TP53- R337H germline mutation). Two of them had first-degree relative with cancer. In our cohort, 22 (33%) patients were stage IV, 14 (21%) stage III, 19 (29%) stage II, 6 (9%) stage I and 5 (7%) unknown stage. 36% of tumors were considered functional (cortisol and/or androgens). At 50 months the estimated OS was 25,9% in the metastatic group and 83% in the localized group (p<0.0001). Among localized patients, 13 (29%) received adjuvant mitotane and 31 (70%) were followed without mitotane. The 12-month DFS rate was 50,8% in the mitotane group versus 67,8% in the group without mitotane (p = 0.83). The Ki67 index was performed in 20 localized patients. The 12-month DFS rate was 58,2% in the high Ki67 index (>10%) group versus 75% in the low ki67 index (≤10%) group (p = 0.47). In metastatic scenario 31 (47%) underwent systemic treatment and the most common (71%) first line chemotherapy was EDP (etoposide-doxorubicin-cisplatin) plus mitotane with an objective response rate of 27% and 56% of them had progression of disease as the best response to treatment. 14 patients received second line and 7 received third line treatment. Gemcitabine-capecitabine,cisplatin-based and Pembrolizumab were the most common regimens used in advanced lines. Conclusions: ACC has a poor prognosis and a significant prevalence of personal and family history of cancer. EDP-mitotane was the preferred regimen used as the first line treatment. As a rare disease, worldwide data can add evidence for future improvements in adrenal cancer.