The present paper reports on a family (2 parents and 3 children) with a minor hereditary polydysplasia: lentiginosis on the face or body, psychic disturbances (oligophrenia, psychopathy) and behavioral troubles, dysraphia (with enuresis up to the age of 8–16 years in the children), strabism and dyscrinism. The EEG was altered in 3 of 4 cases. The three caryotypes performed were normal. The mode of transmission was dominant. The authors review the observations of lentiginosis, published in the medical literature; there is almost always an association with other developmental anomalies; sometimes there are also neuropsychical disturbances. Therefore, the lentiginosis can be generally considered as the cutaneous signature of polydysplasic, often heredo-familial syndrome