Oncocytic neoplasms are most rarely found in the adrenal gland. They are usually benign and non-functioning. We present a case of a testosterone-secreting oncocytic adrenocortical carcinoma in a 19-year-old female who presented with acne, hirsutism and irregular menses. Clinical investigations revealed an elevated testosterone and DHEA-S and a 4×5 cm left adrenal mass. The tumour was successfully excised. The histology showed the tumour to be comprised of oncocytic cells with granular, eosinophilic cytoplasm, features consistent with an oncocytic carcinoma. This is the first case presented of a testosterone-secreting oncocytic adrenocortical carcinoma.

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