Leydig cell tumour (LCT) is a stromal testicular tumour comprising 3% of testicular neoplasms. Metastases are rare, have a poor prognosis and can appear many years after tumour removal. Current therapy for metastatic disease is limited, with no role for radiotherapy and poor efficacy of chemotherapy regimens. About 5070% of metastatic LCT show associated steroid excess, comprising not only androgens but sometimes steroids physiologically produced in the adrenals. Here we report the case of a 51-year-old man who presented with disseminated metastatic LCT deposits in liver, lung, and retroperitoneum after an orchidectomy 15 years previously. His serum testosterone was highly elevated at 93 nmol/l (normal 729); his 24-h urinary androgen metabolite excretion (androsterone+etiocholanolone) was 101 476 μg/24 h (normal 14877957). This was associated clinically with significantly impaired well-being due to restlessness, insomnia, reduced concentration, increased aggression, redness of the face and increased body hair growth. Prognosis was assessed as poor and following detailed discussions the patient opted against chemotherapy. In a palliative approach we decided to initiate the adrenolytic agent mitotane in an attempt to improve the testotoxicosis. His clinical signs and symptoms improved within a few weeks of treatment initiation and prior to reaching therapeutic mitotane levels (1420 mg/l after 5 months of treatment); he returned to full-time work and enjoyed normal quality of life. CT follow-up imaging 6 months after starting mitotane showed stable disease. Concurrently, his urinary androgen metabolite excretion dropped from 101 476 to 12 827 μg/24h. However, after 10 months of mitotane treatment he died suddenly of a suspected cardiac arrest. Review of the literature identified four previous reports on mitotane therapy in LCT for >2 months, all reporting tumour response and reduction in steroid excess. LCT cells may share steroidogenic properties with adrenal cells and thus mitotane treatment for metastatic LCT appears to be a valid palliation option.