AVM formation was traditionally theorized as congenital or acquired in early childhood, but recent hypotheses have emerged considering de novo AVM formation in adulthood. This requires two hits: an inherited genetic mutation followed by an environmental trigger later in life which is commonly a disease state that leads to a vascular insult. However, there have only been 2 reported cases of AVM formation in the setting of liver hepatopathy alone. In these cases, venous hypertension and thrombosis served as the necessary ‘second hit’ in the pathway of AVM formation. In the setting of liver cirrhosis, pro-angiogenic factors released from the liver such as TGF-beta, TNF-alpha, VEGF, IL-6, matrix metalloproteinases, or deficient hepatic estrogen metabolism contributed to this phenomenon.