Leiomyomas are benign uterine tumors and they are the most common tumor of female genital tract. ‘Cotyledonoid myoma’, also known as ‘Sternberg tumor’, is a rare type of leiomyoma and has a unique nature with surgery as the main step in its treatment. The differential diagnosis of cotyledonoid myoma from a malignant condition is important for the follow up of patients. Here we present the clinical evaluation of a 46 years-old patient with cotyledonoid myoma.