“…Andrews [1] describes the occurrence of hematemesis soon after birth in patients in which the characteristic pigmentation has de veloped only at the age of 5. On the other hand, the first symptoms may also occur in aged patients [26], In one third of cases, the symptoms appear before the 10th birthday; in another third abdominal symptoms ap pear around the age of 20. The sudden appar ition and variability of the symptoms may lead to a diagnosis of Peutz-Jeghers syn drome only following surgery ( fig.…”