ABSTRACT
Rhabdomyosarcoma [RMS] is the single most common type of soft tissue sarcoma in children and adolescents but it is extraordinarily rare in neonates. Extremity RMS comprises 20% of all sites, occurs more commonly in the leg than in the arm and accounts for 9% of all RMS cases. According to our review, this is the second case of RMS on day one of life with congenital, antenatal feature, and postnatal progressive clinical course of a large tumor of the hand [pretreatment staging T2bN1M0] with embryonic histological subtype and unfavorable prognosis. The patient is a term newborn boy with huge mass in the right hand and palpable lymph node in subaxillar region. Congenital embryonal rhabdomyosarcoma is a rare form of sarcomas with congenital in nature, antenatal feature and post natal progressive clinical course of sarcomas of extremities in newborn infants