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A composite review of 24 cases of embryonal rhabdomyosarcoma which is seen in fact to be the commonest orbital malignant tumour in children. In its evolution, most often very rapid, sometimes pseudo-inflammatory, this tumour presents many different histological appearances among which the alveolar form and the botryoidal form should be particularly distinguished. Although it was for a long time confused with reticulosarcoma the tumour is in fact derived from primitive undifferentiated mesenchymal cells and can therefore in this sense be considered as a blastoma. Since the prognosis is grave and its treatment often disappointing, it should in many cases combine surgery with radiotherapy and polychemotherapy.
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