Mazabraud's syndrome, though uncommon, is reported increasingly freque
ntly. It represents an entity readily recognisable radiologically on M
R imaging. Awareness of the syndrome, particularly when the myxoma is
solitary, can prevent misdiagnosis of intramuscular myxomas (especiall
y when large) as malignant mesenchymal tumors containing myxoid tissue
. We review the 34 cases previously reported in the literature and inc
lude a recent case from our center.