Rhabdomyosarcoma is the most common type of soft tissue sarcoma in children. The authors present a rare case of eyelid rhabdomyosarcoma in a newborn, who was found to have a reddish eyelid tumor in his OD. A mass with a clear margin, confined to the upper eyelid, was revealed using orbital MRI. Intralesional steroids were injected under the impression of a capillary hemangioma and the tumor shrank initially, but grew rapidly later. Therefore, a debulking surgery was performed and the final diagnosis was embryonal rhabdomyosarcoma. After the operation, metastases still occurred despite the treatment with chemotherapy and concurrent radiation. The patient expired at 6 months of age. In an autopsy, a neuroblastoma was incidentally found in his left adrenal gland. Early biopsy may help lead to an early correct diagnosis and avoid metastases in similar cases.