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Avascular necrosis of bilateral femoral heads in a patient with Fabry's disease.The underlying cause of avascular necrosis (AVN) of the femoral head is often not apparent. We report the case of a 26 year old builder with a four month history of bilateral hip pain, and a diagnosis of bilateral femoral head avascular necrosis. Fabry's disease was identified as the probable cause. Since 2001, enzyme replacement therapy for Fabry's disease has become available, with a potential to influence the disease process, and this is of potential importance to clinicians treating AVN.