Primary ovarian leiomyoma is a rare benign ovarian tumor. Ovarian leiomyoma accompanied with atypical Meigs' syndrome is extremely rare. A 13-year-old woman had underlying primary lymphedema and visited our clinic due to associated vulvar edema. During a gynecologic examination, we detected a right ovarian tumor with large amounts of ascites on transrectal ultrasonography. The tumor size increased from 4 to 7 cm during 6 months follow-up. After laparoscopic right ovarian tumorectomy, the final pathology of the tumor was primary ovarian leiomyoma. Ovarian leiomyomas are not typically suspected before surgery due to their extreme rarity and because they are easily misdiagnosed as fi broma during frozen biopsy due to similarities between both diseases. Here we introduce the case of a primary ovarian leiomyoma accompanied with atypical Meigs' syndrome.