[The leopard syndrome, a cardio-cutaneous syndrome]

A patient with the "leopard" syndrome presented with cardiomyopathy and a large arteriovenous shunt of the left renal vessels. The skin manifestations included multiple lentigines, junctional naevi, blue naevi, and one malignant lentigo. This syndrome is a rare entity, but in all patients with multiple lentigines the possibility of associated cardiovascular changes should be considered. In addition, regular monitoring of the pigmented lesions should be performed because of the increased risk of development of malignant melanoma.