Rhabdomyosarcoma is the most frequent malignant tumour of the orbit in the pediatric age. The chances of survival of patients affected by such a highly malignant neoplasia have significantly improved in recent years. The results obtained up until now with various therapeutic approaches are reviewed briefly and the results obtained in four children affected by rhabdomyosarcoma of the orbit are reported who were treated at the Ophthalmology and Pediatric Clinics of Siena University. The possibility is advanced that the less aggressive and a cautiously conservative treatment can produce the equivalent excellent results which are presently obtained by very destructive therapeutic approaches.