Primary desmoplastic small round cell tumor of the kidney in a 7-year-old girl.

Desmoplastic small round cell tumor (DSRCT) is a rare pathologic entity that most frequently affects the peritoneal cavity and presents in adolescent boys. It often presents at an advanced stage and has a generally poor prognosis. We present an unusual case of DSRCT of the kidney presenting in a young girl. This is the first report of DSRCT of the kidney in the pediatric population, and it suggests that this condition should be a diagnostic consideration when assessing a child with a kidney mass.