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The annual incidence of a first episode of deep vein thrombosis or pulmonary embolism (PE) in the general population is 120 per 100,000. Cancer is associated with an approximately 4- to 7-fold higher risk of thrombosis. Adrenocortical carcinoma (ACC) is a rare type of malignancy, accounting for 0.02% of all cancers reported annually. Approximately 40% of ACCs are nonsecretory. Most patients with nonsecreting tumors have clinical manifestations related to tumor growth (e.g., abdominal or flank pain). Often the adrenal mass is detected by chance via radiographic imaging. As a result, most ACC patients are diagnosed at an advanced stage and have a poor prognosis. Herein, we report a case of a 54-year-old woman who was admitted to our emergency department complaining of dyspnea. She was diagnosed with ACC accompanied by thrombi in the pulmonary artery and inferior vena cava. We performed a left adrenalectomy and administered adjuvant radiotherapy. The patient is currently receiving warfarin and adjuvant mitotane therapy. She was incidentally diagnosed with ACC, with PE as the initial manifestation.