Multiple cardiocutaneous syndromes and associations are described and reviewed with the emphasis on syndromes that are likely first to be recognized by dermatologists, thereby creating awareness of potential significant cardiovascular disease. Multiple lentigines syndrome, the Carney complex, and Marfan syndrome are examples of syndromes with embryologic and genetic overlap between the cardiac and cutaneous systems. An exhaustive, complete listing of every known disorder or syndrome with combined cardiac and cutaneous findings is beyond the scope of this review. However, all the common associations, both acquired and inherited, including connective tissue, autoimmune, infiltrative, and endocrine disorders are discussed. Because of the serious nature of many of the cardiac defects, early recognition of these conditions by dermatologists and appropriate referrals to colleagues is essential; in addition, when appropriate, genetic counseling is recommended for known heritable disorders. (J Am Acad Dermatol 2002;46:161-83.) Learning objective: At the conclusion of this learning activity, participants should be familiar with the embryology of the neural crest, specifically the development of melanocytes and the heart with its vessels, leading to shared abnormalities of the two systems; the major syndromes and associations producing both cutaneous changes, especially of the pigment systems, and cardiovascular abnormalities; the role of the dermatologist in recognizing these changes; and the need to be cognizant and alert in helping screen for associated systemic disease.