Rhabdomyosarcoma is the single most common type of soft tissue sarcoma in childhood and teenage, it has been reported from birth to the seventh decade, with the majority of cases presenting in early childhood but it is very rare in neonates. The orbit including the eyelids is the usual primary site in the head and neck, and it is a highly malignant tumor. There have been only a few cases of congenital orbital rhabdomyosarcoma previously reported in the literature. We report a case of a newborn girl that was admitted in our structure 2 days after her birth with giant right orbital mass inducing proptosis that was discovered at birth. MRI showed a poorly defined mass but biopsy of the tumor confirmed the diagnosis of Rhabdomyosarcoma. Chemotherapy was started accordingly with VAC regimen with a good evolution under treatment infortunatly the patient died at four month old from an infectious disease because she doen't receive any vaccination. Congenital RMS has a poor prognosis and must benefit from a multidisciplinary approach. Children with cancer need to be immunized against the common vaccine-preventable diseases sometimes during ongoing chemotherapy to increase their chance of survival. Prenatal diagnosis may also improve the prognosis of these patients.