Malignant rhabdoid tumors (MRT) are uncommon, highly aggressive tumors arising usually from the central nervous system and kidneys. Nonrenal and noncentral nervous systems MRT are rare in neonates. To the best of our knowledge, only five cases of congenital MRT of neck have been described till date. We present a rare case of congenital MRT of the neck in a neonate along with review of literature.
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