Rhabdomyosarcoma is a malignant mesenchymal tumor of childhood. The eyelid localization is unusual. We report the case of a 7-year-old child with upper palpebral rhabdomyosarcoma revealed by an isolated blepharoptosis. The CT-scan revealed a well delimited enhancing soft tissue mass involving the upper eyelid. The tumor was excised totally by an upper eyelid incision under general anesthesia. It was limited in preseptal eyelid without extension to the orbit. Histopathologic examination confirmed the diagnosis of embryonic rhabdomyosarcoma. Postoperatively, the upper eyelid regained its motility. The visual axis was cleared. After surgery, three cures of chemotherapy including ifosfamide, vincristine, and actinomycin were administered. After a 4 months follow up period, there was no sign of tumor recurrence.