Malignant rhabdoid tumor (MRT) is an uncommon aggressive neoplasm whic
h usually occurs in the kidney of children, but it has also been found
in extrarenal sites. MRT arising in the vulva is extremely rare. Only
four cases of MRT of the vulva have been reported in the English lite
rature. We herein present another case. The diagnosis and management o
f vulva MRT are reviewed.