A 75-year-old woman presented with a ganglion-like nodule on the dorsa
l aspect of the right foot. A 2.5 x 1.5 cm, saccular and malleable tum
or, that was in continuity with the dorsal venous arch, was completely
resected. It was characterized by a diffuse intramural and circumfere
ntial, low grade, malignant, smooth muscle proliferation with an aneur
ysmal-like luminal space. No endoluminal or periadventitial invasive n
eoplastic component was present. The patient had no evidence of diseas
e at 58-month follow-up. This is the first reported case of venous lei
omyosarcoma in the foot. Furthermore, the intramural confinement of ne
oplastic growth is a unique observation.