Benign symmetric lipomatosis(BSL) is a rare disease characterized by the presence of multiple, symmetric and nonencapsulated fat masses in the face, neck and other areas. It is commonly seen in middle-aged Caucasian Mediterranean males, while its etiology is still not clear. The majority of the patients with BSL have a history of alcohol abuse and hepatopathy. BSL of the limbs is very rare. This article reports a unique case of a 60-year-old Chinese woman with involvement of the knees confirmed by the results of magnetic resonance imaging(MRI) and histopathology, which was not described previously in published literatures.