Coats plus syndrome, also known as cerebroretinal microangiopathy with calcifications and cysts, was a rare autosomal recessive condition caused by a mutation in the CTC1 gene, which codes for conserved telomere maintenance component 1. Aside from retinal involvement, abnormalities in the brain and osteopenia were seen, and life-threatening severe gastrointestinal bleeding and portal hypertension. A 6-year-old child with Coats plus syndrome was sent to the pediatric emergency room after vomiting blood and passing blood in her stool. An upper and lower gastrointestinal endoscopy showed esophageal varices and vascular telangiectasia in the pyloric antrum, duodenum, and colon. After receiving intravenous octreotide, the patient received palliative care, and the bleeding was stopped. Later the patient was seen in pediatric gastrointestinal, neurology, and ophthalmology clinics. They were later admitted to the intensive care unit after her gastrointestinal system continued to bleed intermittently. The patient died as a result of severe gastrointestinal bleeding. Coats plus syndrome could cause gastrointestinal bleeding and portal hypertension, which could be fatal. Because Coats plus syndrome was so uncommon, there wasn’t much information available about it. A case of Coats plus syndrome, an unusual cause of gastrointestinal bleeding and portal hypertension, was presented in the study.