Ascites, pelvic mass and elevated CA-125 in females carry a grim prognosis, likely an ovarian carcinoma. However, more benign etiologies such as Meigs' and pseudo-Meigs' syndrome must be considered. Pseudo-Meigs' syndrome presenting with an elevated CA-125 is rare and presents a diagnostic challenge. Medline and PubMed were queried for pseudo-Meigs' syndrome cases. We present a 35-year-old female patient who presented with abdominal swelling and weight gain. Imaging demonstrated a 29-cm large intraabdominal mass with significant ascites with elevation of CA-125. Surgical resection was performed, and pathology identified uterine leiomyoma. Twenty-one cases of pseudo-Meigs' syndrome were identified in the literature. Most patients presented with abdominal distention, and some also reported dyspnea. All patients, including our case, were treated surgically. No recurrence reported among these cases. Surgery is the mainstay for radical treatment in pseudo-Meigs' syndrome. Resolution of the ascites and hydrothorax occurs following resection of the tumor.