Adrenal myelolipoma is an unusual, benign and hormonally inactive tumour composed of mature adipose and haematopoietic tissue. In the past, these tumours were accidentally discovered at autopsy. Today, they are found much more frequently and incidentally, mainly because of the widespread use of ultrasonography, computed tomography and magnetic reasonance imaging. Adrenal myelolipoma is usually unilateral and asymptomatic. The case of a 52-year-old female patient, surgically treated for a symptomatic giant myelolipoma of the right adrenal gland, is reported, together with a review of the literature.