Abstract
Congenital extrarenal rhabdoid tumor is a rare soft-tissue tumor, with the most common sites of origin in the genitourinary tract, head and neck regions, and extremities are less commonly involved. Malignant rhabdoid tumors are highly aggressive tumors, and when they develop in utero, the prognosis is even worse, with almost no reports of survival beyond the neonatal period. We present a rare case of congenital extrarenal extracranial rhabdoid tumor on the right shoulder reported at birth and later died after excision due to recurrence of the tumor with distant metastasis.