Peutz-Jeghers syndrome, first described in 1921 by Peutz and later on, in 1949, by Jeghers, is a relatively rare condition characterized by the association between cutaneous-mucosal pigmentation alterations with a specific distribution and multiple hamartomatous colonic polyps with an onset during the first 10 years of life and with a 10- 18 times higher risk of malignant degeneration than that of the general population [1,2,3,4,5,6,7].
Clinically, Peutz-Jeghers syndrome is characterized by cutaneous lesions consisting of brown-black or bluish pigmented macules similar to ephelides that are predominantly distributed on the cephalic extremity, as well as on the palmo-plantar and perianal regions, and are associated with gastrointestinal disorders (gastrointestinal hamartomatous polyps, occlusions and intestinal intussusceptions) and various neoplasms (digestive, thyroid, breast, genital and lungs) .
Taking into account the multiorgan involvement and the increased risk of severe complications, the multidisciplinary approach of these patients is essential for therapeutic success.
In the present paper we present the case of a 53-yearold patient who requested a dermatological consultation for multiple pigmented labial macules of brownish-black color, affirmatively evolving since childhood. Anamnestically, the patient stated irregular bowel habits with diarrheaconstipation alternations, so that the patient was referred to the general surgery service, where a laparoscopic sigmoidectomy was performed for multiple suspiciouslooking polyps that were discovered colonoscopically. The histopathological examination of the biopsy material established the diagnosis of Peutz-Jeghers syndrome.