AbstractXanthogranulomatous inflammation is an uncommon, nonneoplastic, chronic process in which the affected organ is destroyed by massive cellular infiltration of foamy histiocytes admixed with multinucleated giant cells, plasma cells, fibroblasts, neutrophils, and foci of necrosis. The organs most commonly affected by this entity are kidney and gallbladder. Only a few cases involving the ovary have been reported. The etiology of this entity is unknown, but the associated risk factors are infection, inappropriate antibiotic therapy, use of intrauterine contraceptive device, abnormalities in lipid metabolism, endometriosis, leiomyoma, and recurrent pelvic inflammatory disease. The current case was a 31-year-old female with post-in vitro fertilization (post-IVF) twin pregnancy, who presented with pain in the abdomen, provisionally diagnosed as ovarian mass with suspicion of malignancy. Patient was treated with antibiotics and an elective lower segment cesarean section was planned, due to lower abdominal pain and the presentation of both fetuses being breech. Patient underwent exploratory laparotomy with right oophorectomy and left double-J stenting, in view of left hydronephrosis. Diagnosis of xanthogranulomatous oophoritis was established on histopathological examination. There is only a single case; previously documented from India, in the setting of primary infertility. The present case report stresses that the condition may affect women of any age group and may be encountered in pregnancy, as has been observed in this case. Hence, thinking about this differential diagnosis in the cases presenting with similar complaints, would be of great value to avoid misdiagnosing them.