eng Science and Education Publishing American Journal of Medical Case Reports 2374-216X 2023-08-13 11 8 142 145 10.12691/ajmcr-11-8-3 AJMCR20231183 article Anahat Kaur MD 1 Bhanujit Dwivedi MBBS bhanujit.dwivedi@gmail.com 2 Angad Singh MD 3 Tejasvi Dwivedi MBBS 4 Rubina Sharma MD 5 Sherrie White MD 6 Department of Hematology and Oncology, Albert Einstein College of Medicine, Jacobi Medical Center, Bronx, NY, USA Maulana Azad Medical College, New Delhi, India HCA Midwest Healthcare, Kansas City, KS, USA All India Institute of Medical Sciences, Raipur, India PGIMER, Chandigarh, India Department of Pathology, Jacobi Medical Center, Bronx, NY, USA Adenocarcinoma of the small intestine is one of the most uncommon gastrointestinal malignancies, comprising <5% of all GI cancers. Krukenberg tumors are also uncommon, making up only 3-5% of ovarian malignancies. We are reporting a rare case of a patient with small bowel adenocarcinoma presenting with Krukenberg metastasis. A 79-year-old female initially presented with abdominal pain and diarrhea and was found to have a long segment of irregularly thickened distal ileum suspicious for malignancy. The patient was unable to maintain consistent follow-up for almost a year after which she presented with early satiety, abdominal bloating, palpable abdominal mass and 20 lb weight loss. US and CTAP now revealed a large complex cystic mass in the pelvis with persistent bowel wall thickening and elevated CA- 125 tumor marker. The site of origin could not be determined on FNA biopsy so the patient underwent surgical resection with pathology showing adenocarcinoma of the ileum with metastatic involvement of the right ovary (Krukenberg metastasis). Molecular somatic mutation testing showed microsatellite instability (MSI-High status). She completed 12 cycles of consolidative chemotherapy with FOLFOX and currently remains in remission on pembrolizumab maintenance.This case highlights the rarity of presentation, diagnostic challenges and successful treatment of patients with ileal adenocarcinoma presenting with Krukenberg metastasis. http://pubs.sciepub.com/ajmcr/11/8/3/ajmcr-11-8-3.pdf Ileal adenocarcinoma Krukenberg metastasis Debulking surgery Immune checkpoint inhibitor