angiomyolipoma ( aml ) is a benign mesenchymal tumour consisting of varying amounts of mature adipose tissue , smooth muscle , and thick walled blood vessels . it is relatively rare benign tumour appearing in about 0.3% of general population and accounts for 3% of solid renal masses . extra renal amls ( eramls ) are rare tumours that present as incidentalomas upon imaging for other conditions . case reports of extra renal aml are rare with < 60 reported cases since they were first described by friis and hjortrup in 1982 . our case described one of the largest ever reported myolipomas , which despite its size , can be rather asymptomatic . magnetic resonance imaging ( mri ) revealed 9.5 cm  8 cm  2 cm right adrenal mass [ figure 1 ] . the tumour was predominantly hyperintense with signal intensity similar to fat on t1/t2-weighted images and lost signal on the fat - suppressed sequences . a well - encapsulated , firm globular mass separate from the right kidney and no definable adrenal was found [ figures 2 and 3 ] . gross post - operative evaluation of the adrenal mass showed approximately 10 cm  8 cm  2 cm greyish white solid , smooth , and firm mass [ figure 4 ] . histological examination revealed benign adrenal cortical elements , with a predominance of mature benign adipose tissue interspersed with pockets of haemopoietic ( erythro - myeloid ) intermediates , and megakaryocytes which was confirmatory for the diagnosis of adrenal aml . angiomyolipoma is a rare clinical entity mostly involving kidney and is part of the group of tumour known as tumours of perivascular epithelioid cell origin . the most widely accepted theory of origin is adrenocortical cell metaplasia in response to stimuli , such as necrosis , inflammation , infection or stress ( meyer et al . ) . the presence of perivascular epithelioid cells is characteristic of aml since these cells show immunoreactivity for muscle markers ( epithelial membrane antigen , keratin , vimentin , desmin , and actin ) and hmb-45 . positive immunoreactivity for hmb-45 , a monoclonal antibody , is characteristic of amls and can be used to differentiate amls from other similar appearing lesions . one is isolated aml , and the other is that is associated with tuberous sclerosis . most patients of isolated cases are in the age group of 27 - 72 years , mean age being 43 years . although the majority of eramls are benign , two cases of metastatic and recurrent eramls have been reported . the demonstration of fat density ( hypodense ) within an adrenal mass by computed tomography is virtually diagnostic of aml . mri is sometimes required to demonstrate origin of the tumour , to define the tissue planes when the tumour is large and heterogeneous , and to distinguish benign from malignant lesions by comparing signal intensity ratios of adrenal to liver . a small , < 5 cm , asymptomatic myelolipoma could be followed - up over 1 - 2 year period with imaging controls . on the contrary , asymptomatic lesion or a large > 5 cm myelolipoma should be surgically excised , since there are reports of spontaneous rupture and haemorrhage of the mass presented with life - threatening cardiovascular shock . unstable patients may benefit from emergent tumour embolisation and a subsequently staged surgical resection . since it is a benign disease , its prognosis is good . nevertheless , follow - up is recommended because of atypical morphology . currently , there is no agreed protocol on follow - up but an ultrasound three to 6 months following the surgery with annual clinical examination for large tumours is recommended . the adrenal myolipoma is a rare urological entity , which seems to increase its frequency , probably due to causes affecting primarily the function and physiology of the adrenals . ever since its introduction by gagner in 1992 , laparoscopic adrenalectomy has become the standard of care for the treatment of functioning and non - functioning adrenal tumours . many authors have found a decrease in perioperative morbidity and convalescence after this procedure when compared with open surgery .
angiomyolipoma ( aml ) is a rare mesenchymal tumour arising from perivascular epithelioid cells . 
 it is most commonly seen in kidney , but rarely aml can arise in extra renal sites . 
 adrenal aml is a very rare clinical entity , and very few cases have been reported so far . 
 we present our experience with a 43-year - old female , who presented with right flank pain . 
 magnetic resonance imaging showed a right adrenal mass . 
 laparoscopic adrenelectomy was performed , and the histopathology report confirmed the diagnosis of aml . 
 patient was discharged uneventfully .