[Peutz-Jeghers syndrome. Report of a case]

The author describes a case of Peutz-Jeghers' Syndrome, an affection determined by a dominant autosomic gene and which is characterized by pigmentation of the skin and mucosas associated with gastrointestinal polyposis; further it is to be reminded that in distinct pathologies it is also possible to observe the presence of skin lesions and the imperilment of internal organs.