Background: A new cardiocutaneous syndrome has been noted, characterized by an epidermolytic palmoplantar keratoderma and woolly hair, and associated with dilated cardiomyopathy. Objective: This describes the clinical and histopathologic characteristics of this new syndrome. Methods: Eighteen patients were examined clinically and histologically from 1970 to 1997. Cardiologic examinations were performed in 12 patients. The cutaneous lesions were classified according to the presence of obligatory and facultative elements of the syndrome. Results: Patients were born with woolly hair. Around the first year palmoplantar keratoderma and the other keratotic elements appeared. The first cardiac abnormalities are exclusively electrocardiographic and occur in asymptomatic patients. In these patients, dilation of the left ventricle together with alterations in muscle contractility are observed. The dilated cardiomyopathy can lead to congestive heart failure and death. Conclusion: The association of woolly hair and palmoplantar keratoderma with a histopathologic pattern of epidermolytic hyperkeratosis has not been previously described. Their frequent association with dilated cardiomyopathy defines a cardiocutaneous syndrome. Whenever woolly hair is associated with any kind of palmoplantar keratoderma, a search for possible cardiac abnormalities is recommended. (J Am Acad Dermatol 1998;39:418-21.)