Malignant rhabdoid tumor is a rare and highly malignant renal tumor of infa
ncy. Extrarenal tumors involving the orbit have been reported, but never at
birth.(1-5) The authors describe a primary malignant rhabdoid tumor of the
orbit in a neonate who had massive unilateral proptosis at birth. Clinical
, radiographic, and histologic features of the tumor are discussed.