Primary adrenal angiosarcoma is a rare vascular malignancy with fewer than fifty cases reported in the literature, with a preference for middle-aged men and a poor prognosis with a median survival of around eighteen months and a five-year overall survival of 30 %. A case of a 61-year-old male patient who consulted for pain in the left renal fossa radiating to the ipsilateral flank and constitutional syndrome is reported. Diagnostic imaging tests show a large solid left adrenal mass with no signs of local tumor extension or in other organs; Laboratory studies confirm autoproduction of cortisol and association with probable paraneoplastic consumptive coagulopathy. Open left adrenalectomy was performed with an anatomopathological diagnosis of primary epithelioid angiosarcoma of the adrenal gland and liver metastases. During the immediate postoperative period, hematoma of the surgical site was evidenced, which was treated conservatively with favorable evolution. Subsequently, adjuvant chemotherapy treatment with paclitaxel was administered, presenting metastatic progression in diagnostic imaging tests five weeks after surgery. Exitus letalis, presented three months after surgery.
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