Xanthomas are localized cholesterol deposits most observed in cutaneous sites. They serve as the physical indicators of underlying hyperlipidemic states and carry a significance in familial hyperlipidemias. Xanthomas occur in all age groups and are a marker for an atherogenic state and an increase in future cardiovascular events. Some subtypes such as eruptive xanthomas are associated with acute pancreatitis. Xanthomas in unusual sites such as visceral and mucosal areas appear associated with malignancies. Understanding the pathophysiology and early and adequate management of hyperlipidemia can significantly reduce the incidence of vascular events. Xanthomas are treated mostly for cosmetic needs surgically or through laser excision and various other recent modalities to minimize recurrence and scarring. The mainstay of management lies in correcting the underlying hyperlipidemias with diet and lifestyle modifications. The pharmacological approach involves lipid-lowering therapies to prevent future vascular events; they include statins, ezetimibe, emerging monoclonal antibodies, and PCSK-9 inhibitors. Complete removal of xanthomas is achieved by surgical correction or laser therapy.