Intramuscular myxoma is a rare, benign, mesenchymal tumor. The tumor usually occurs in adults between 40 to 60 years old with a predilection for women (70%). The most common sites of tumors are the large muscles of the thigh. It is usually solitary, and multiple intramuscular myxomas are often associated with fibrous dysplasia. Of all diagnostic images, MRI offers the best imaging approach to determine tumor local extension and a differential diagnosis between benign and malignant tumors. However, the accurate diagnosis is always made by microscopic examination. Neither recurrence nor metastasis was seen after local excision. We present a typical case and a thorough literature survery.