Abstract : Peutz–Jeghers syndrome (PJS) is a rare inheritable condition characterized by hamartomatous polyposis of the gastrointestinal tract, with oral mucocutaneous pigmentation. We report the case of a 6-year-old boy who presented with gynecomastia and testicular enlargement. The family history was interesting for different types of tumors from the maternal side. Moreover, the examination revealed incidental peri-oral and buccal lentigines spots. Most endocrine presentations in boys involve testicular tumors, precocious puberty, and tall stature. Regular clinical follow-up is essential to evaluate the intestinal polyps, endocrine function, and tumors in these patients.