A review was done of 250 consecutive biopsies for orbital space-occupying lesions in children. Benign cystic lesions were by far the most common, accounting for 52% of the lesions. Inflammatory lesions, usually biopsied to exclude the diagnosis of rhabdomyosarcoma, accounted for 16%. Among the 250 biopsies there were 14 primary malignant tumors (6%), ten of which were rhabdomyosarcoma. Although rhabdomyosarcoma is not the most common space-occupying orbital lesion in children, clinicians should be familiar with its clinical features and proceed with immediate biopsy followed by appropriate irradiation and chemotherapy if the diagnosis is established. However, biopsy should only be undertaken if there is reasonable suspicion that the lesion could be rhabdomyosarcoma or other childhood orbital malignancy.