Background Peutz-Jeghers syndrome (PJS), is an uncommon autosomal dominant disorder caused by a mutation at 19p13.3 that manifests as mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal system. We report a case of a 19-year-old young girl presented with intermittent episodes of abdominal pain and few occasions of fresh per rectal bleeding as well as features of anemia. Case Presentation A 19-year-old female patient with a urinary tract infection was brought into our internal medicine department. It was discovered that she had mucocutaneous pigmentation and iron deficiency anemia. She also reported having vague stomach ache and a couple instances of hematochezia since she was fifteen. Colonic polyps, colon cancer, or any other gastrointestinal condition were not present in the family history. Physical examination revealed no abnormalities, except for the presence of dark-brown pigmented maculae around the oral mucosa, lower lip and pulp of fingers. Numerous pedunculated polyps were seen dispersed throughout the colon during a colonoscopy. There were a lot of giant polyps. Polyps were seen in the large as well as small bowels during MR enterography. Background Peutz-Jeghers syndrome (PJS), is an uncommon autosomal dominant disorder caused by a mutation at 19p13.3 that manifests as mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal system. We report a case of a 19-year-old young girl presented with intermittent episodes of abdominal pain and few occasions of fresh per rectal bleeding as well as features of anemia. Case Presentation A 19-year-old female patient with a urinary tract infection was brought into our internal medicine department. It was discovered that she had mucocutaneous pigmentation and iron deficiency anemia. She also reported having vague stomach ache and a couple instances of hematochezia since she was fifteen. Colonic polyps, colon cancer, or any other gastrointestinal condition were not present in the family history. Physical examination revealed no abnormalities, except for the presence of dark-brown pigmented maculae around the oral mucosa, lower lip and pulp of fingers. Numerous pedunculated polyps were seen dispersed throughout the colon during a colonoscopy. There were a lot of giant polyps. Polyps were seen in the large as well as small bowels during MR enterography. © 2018 American Medical Association. All rights reserved. Discussion: PJS syndrome is incredibly rare; babies with an estimated incidence of 1:8,500 to 1:200,000 are affected. The young lady in our presentation met the inclusion criteria for the syndrome since she had mucocutaneous pigments on her hands and lips in addition to many Peutz-Jeghers polyps in the small the small and large intestine. Approximately 95% of PJS patients have mucocutaneous pigmentations, which may be the initial clinical clue prior to the onset of gastrointestinal symptoms. When a kid has iron deficiency anemia, which is common in youngsters, it is crucial to perform a complete physical examination and look into the family history. This enables the early detection of uncommon illnesses like Peutz-Jeghers syndrome. PJS patients have a cancer incidence that is almost fifteen times greater than the normal population. This case emphasizes the value of routine surveillance, patient education, and tertiary prevention strategies in preventing complications in individuals with post-diagnosed Puetz-Jeghers syndrome.