Background: Hemorrhage from an angiomyolipoma (AML) of the kidney can be life threatening and arterial embolization is the primary treatment. Embolization is less invasive than surgery, is well tolerated, and major complications are rare. We describe a case of disseminated intravascular coagulation (DIC) after embolization of a bleeding renal AML in a 44-year-old man with massive bilateral AMLs. This report aims to highlight the possibility that acute DIC could be a major complication of embolization itself and so should be considered and screened for because, if present, it requires early and aggressive management. Case Presentation: A 44-year-old man with a history of large bilateral renal AMLs associated with tuberous sclerosis complex presented with visible hematuria and abdominal pain. Renal CT revealed bleeding from the right kidney. Embolization with polyvinyl alcohol and lipiodol was urgently performed. The following day he required multiple blood transfusions and repeat embolization, this time with gelfoam and "tornado" coils. He suddenly developed DIC, cardiovascular collapse and acute renal failure requiring many days in the intensive care unit for inotropic support and renal replacement therapy. Conclusion: Arterial embolization may be associated with increased risk of DIC in the setting of treating large bleeding renal AMLs. DIC may be a direct or indirect complication of this. The clinician must act quickly to identify this and treat this complication aggressively.