Fanconi's Anemia is primarily an autosomal recessive genetic disorder 
characterized by congenital abnormalities, defective haematopoiesis leading to 
bone marrow failure and increased risk of development of Myelodysplastic 
syndrome, acute myeloid leukemia and solid tumours. Chromosomal instability can 
be demonstrated by breakage caused by alkylating agents and forms the basis of 
diagnosis. Our patient presented with structural deformities associated with 
features of bone marrow failure in form of pancytopenia. Bone marrow analysis 
and flow cytometry done on aspirate was suggestive of MDS. He subsequently 
progressed to frank acute myeloid leukemia and succumbed to the illness. The 
case is being reported for its rarity especially, Fanconi's Anemia associated 
with monosomal karyotype (one monosomy plus one more structural abnormality).