Extra-adrenal myelolipomas are rare, benign tumors composed of hematop
oietic and adipose elements. Although these tumors can cause local sym
ptoms or hemorrhage, they are generally asymptomatic. However, when di
scovered intraoperatively, they pose a diagnostic dilemma to the urolo
gic surgeon. We present a case of perirenal extra-adrenal myelolipoma
discovered intraoperatively in a patient with von Hippel-Lindau diseas
e undergoing partial nephrectomy.