Rhabdomyosarcoma (RMS) is the most common primary malignant tumor of the orbit in childhood. The average age of onset is around seven years. In the present paper, the authors present the clinical and radiological findings in two newborns with histopathologically confirmed orbital rhabdomyosarcoma. A review of the literature was also carried out. In both children, the first sign leading to a visit was a unilateral proptosis, without symptoms or any other sign. Both underwent a CT-scan and an MRI. Confirmation of the histology was made by an open sky biopsy. The children were first treated by chemotherapy but had incomplete resolution of the tumor after more than 11 months for the first case and 9 months for the second. Since the parents of both children refused exenteration, radiotherapy had to be resorted to because of the incomplete response to chemotherapy. Our therapeutic approach and the outcome of these two cases are discussed.