Angiomyolipoma (AML) is a benign tumour of the kidney, with prevalence of 0.28% in males and 0.6% in females; tumours larger than 4 cm are often associated with haemorrhage. The tumour cells highly expresses estrogen receptor; however the functionality of this receptor is uncertain. This case reports a giant angiomyolipoma leading to fatal exsanguination in an obese woman with multiple ovarian follicular cysts and hyperthecosis. This article aims to provide documentation on the effects of hyperoestrogenic state on AML. Case report: A 44-year-old Malay woman was found dead at home and brought to the mortuary of Hospital Kuala Lumpur, Malaysia for post-mortem examination. She had complained of severe left abdominal pain one day prior to death. There was no significant past medical history. Post mortem revealed a body mass index of 54 kg/m2. One litre of blood was found in the peritoneum and her left kidney was effaced by a tumour measuring 24x20x16 cm and weighing 2.8 kg. Blood clot was seen attached to the tumour surface. Both ovaries were found to be enlarged, with presence of multiple cysts measuring 1 to 2 cm. Histological examination of the renal mass showed angiomyolipoma. The ovary showed multiple follicular cysts with hyperthecosis noted in one. This case provides documentation of an adverse outcome of an AML in hyperestrogenic state. It supports the idea of functioning estrogen receptors in these tumours.