Multiple lentigenes syndrome is a complex genetic dermatosis with dominant autosomal transmission an varying degrees of penetration first described in 1969. Apart from disseminated lentigenes at least two of the following are present: cardiac and neurosensory alterations, cervicofacial genitourinary and endocrinological dysmorphias, retarded growth and other skin conditions. MLS has also been called LEOPARD syndrome (Gorlin et al. 1969), thus being an acronym that memorizes the various anomalies involved: L = lentigines, E = ECG anomalies, O = ocular hypertelorism, P = pulmonary stenosis, A = anomalies of the genital organs, R = retarded growth, D = deafness. The clinical and diagnostic aspects of MLS are critically analysed and the case of a woman with the syndrome admitted to San Camillo Hospital, Rome is examined.