Abstract Introduction <p>Gastrointestinal hemangiomas are rare benign vascular tumors that can occur as solitary or multiple lesions throughout the gastrointestinal tract. Multiple gastrointestinal hemangiomas are often associated with syndromic conditions, but their co-occurrence with both celiac disease and lymphangiectasia has been rarely described. In this case report, we present a unique constellation of clinical findings in a female patient with multiple hemangiomas involving the entire gastrointestinal tract, accompanied by Marsh 1 celiac disease and lymphangiectasia, along with homozygous mutations in the MTHFR and PAI-1 genes. To the best of our knowledge, such a combination of features has not been previously reported in the existing literature.</p> Case Presentation <p>A 27-year-old female patient presented with chronic fatigue and severe iron deficiency anemia persisting for 3 years. Laboratory tests revealed hypochromic microcytic anemia, consistently low ferritin levels, and elevated homocysteine levels. Genetic testing showed homozygous mutations in MTHFR (A1298C) and PAI genes, as well as HLA-DQ2.5 positivity. Endoscopic examinations revealed hypervascular polypoid lesions throughout the stomach, duodenum, small intestine, and colon. These lesions were prone to bleeding and were managed with endoscopic polypectomy using Ankaferd hemostatic powder (AHP) for bleeding control.</p> Conclusions <p>This case highlights a rare combination of gastrointestinal and genetic findings, including multiple hemangiomas, lymphangiectasia, and early-stage celiac disease, in association with homozygous MTHFR and PAI gene mutations. While further studies are needed to determine any causal relationships, this constellation of features may inform the diagnostic and therapeutic approach to similar complex presentations.</p>