Fanconi anemia is a rare inherited disease characterized by congenital 
anomalies, growth retardation, aplastic anemia and an increased risk of acute 
myeloid leukemia and squamous cell carcinomas. The disease is caused by mutation 
in genes encoding proteins required for the Fanconi anemia pathway, a response 
mechanism to replicative stress, including that caused by genotoxins that cause 
DNA interstrand crosslinks. Defects in the Fanconi anemia pathway lead to 
genomic instability and apoptosis of proliferating cells. To date, 13 
complementation groups of Fanconi anemia were identified. Five of these genes 
have been deleted or mutated in the mouse, as well as a sixth key regulatory 
gene, to create mouse models of Fanconi anemia. This review summarizes the 
phenotype of each of the Fanconi anemia mouse models and highlights how genetic 
and interventional studies using the strains have yielded novel insight into 
therapeutic strategies for Fanconi anemia and into how the Fanconi anemia 
pathway protects against genomic instability.