Leiomyosarcoma of the adrenal gland is a very rare tumour. We report a case of this rare tumour and review the previous case reports. The patient, a 68-year-old woman, presented with a 1-week history of right loin pain and fever associated with loss of weight and appetite. Computerised tomography and ultrasound scans showed the presence of a right adrenal gland tumour, which was subsequently surgically removed. Histological examination of the 12.5-cm tumour revealed a leiomyosarcoma of the adrenal gland. The patient was alive and well with no evidence of recurrent disease 1 year later.