An autosomal dominant disorder of lipid metabolism. It is caused by mutations of APOLIPOPROTEINS B, main components of CHYLOMICRONS and BETA-LIPOPROTEINS (low density lipoproteins or LDL). Features include abnormally low LDL, normal triglyceride level, and dietary fat malabsorption. The disease is familial apolipoprotein B hypobetalipoproteinemia (Experimental Factor Ontology id EFO_1001789). Also known as: Hypobetalipoproteinemia, Familial, Apolipoprotein B.