Ovarian leiomyoma is a rare, benign spindle cell tumor with a favorable prognosis. Myxoid leiomyoma, a rare subtype of leiomyoma, exhibits significant myxoid changes. We present a case study of a rare ovarian myxoid leiomyoma in a young woman. The patient presented with left flank pain and a large abdominal mass upon physical examination. Imaging indicated a solid‐cystic right ovarian mass. The patient underwent a right salpingo‐oophorectomy, and a 5 kg mass with myxoid degeneration was resected. Histopathological and immunohistochemical examination confirmed the diagnosis of ovarian myxoid leiomyoma. We discussed the challenges in the diagnosis of ovarian myxoid leiomyoma and emphasized the importance of accurate diagnosis and appropriate surgical treatment, with a favorable prognosis. This case contributes to the understanding of rare ovarian tumors and highlights the significance of histopathological and immunohistochemical examination in confirming the diagnosis.