Introduction:Primary adrenal epithelioid angiosarcoma is an exceptionally rare condition, with only 51 cases reported in the literature since 1988.Case presentation:We report a case of a 59-year-old male patient who presented with a 4-month history of left flank pain and anemia. Radiographic imaging identified a 14 cm mass lesion in the left suprarenal region, which showed heterogeneous enhancement. The patient underwent total adrenalectomy with tumor-free surgical margins. Histological sections showed features consistent with an epithelioid tumor, and immunohistochemical staining confirmed the diagnosis of epithelioid angiosarcoma of the left adrenal gland.Discussion:Primary adrenal epithelioid angiosarcoma is a very rare entity. It was first described by Karetiet al.in 1998. The most common presentation is an abdominal mass associated with pain. As there are no specific imaging findings for this tumor, histology combined with immunohistochemistry is the most definitive diagnostic method. Surgery with adjuvant chemotherapy is the management reported for previous cases.Conclusion:In cases of rare malignancies, interdisciplinary collaboration is crucial for determining the optimal management strategy.