Lomitapide Mesylate is primarily used as a lipid-lowering agent for the treatment of homozygous familial hypercholesterolemia (HoFH), a rare genetic disorder characterized by extremely high levels of low-density lipoprotein cholesterol (LDL-C). It works by inhibiting the microsomal triglyceride transfer protein (MTP), which is essential for the assembly and secretion of very low-density lipoproteins (VLDL) in the liver. By reducing VLDL production, it significantly lowers LDL-C levels in patients who have limited treatment options. It is typically prescribed as an adjunct to other lipid-lowering therapies, including low-fat diet and LDL apheresis, for patients who cannot achieve target cholesterol levels through conventional treatments. Due to its potential for causing liver toxicity, its use is closely monitored, and liver function tests are regularly performed during therapy. It is also contraindicated in pregnant women due to the risk of fetal harm.