Background: Ovarian edema, ovarian leiomyoma, and double inferior vena cava are all rare clinical entities. The coexistence of all these entities has not been yet reported in the literature. Case presentation: We report a case of a 25-year-old nulliparous tamang woman with all these rare clinical entities, who presented with a complaint of right-sided lower abdominal pain. After examination and investigation, an ovarian tumor was suspected and laparotomy was performed during which bilateral ovarian edema with a solid tumor on the left side was identified and left salpingo-oophorectomy was done preserving her right ovary. A histopathological examination confirmed the clinical findings. Conclusions: As ovarian edema is a rare entity, due to lack of clinical suspicion it is often overdiagnosed as a malignant tumor leading to radical surgery with subsequent loss of hormonal function and early infertility. A high degree of clinical suspicion during the intraoperative period is helpful for diagnosis to avoid unnecessary oophorectomy and infertility.