SUMMARY
Clinical and histopathologic features of one case of orbital alveolar soft part sarcoma in a 16-month-old girl with unilateral exophthalmos is reported. A biopsy was performed showing material compatible with alveolar soft part sarcoma. The child died of respiratory arrest and the necropsy confirmed the tumor as a primary left orbit alveolar soft part sarcoma extensive to the great sphenoidal wing and sella turcica. Metastasis were detected to both lungs. The present report amphasizes the need to consider this rare neoplasm in the differential diagnosis of orbital tumor, either in children as in adults.