Wang Y, et al. - Researchers sought to describe the related clinicopathologic factors, treatment, and prognosis of recurrent orbital alveolar soft part sarcoma (ASPS; a clinically and morphologically distinct malignant soft tissue tumor) in children. Unilateral proptosis, restricted ocular motility and impaired visual acuity of the affected eye were the presenting complaints of three children aged from 1 to 12 years. All cases exhibited periodic acid Schiff-positive diastase-resistant crystalline granules. The tumor cells of two patients had positive results for immunostaining of TFE-3, INL1, and Ki67. Patient 2 and patient 3, who only underwent tumor resection, had a shorter time until local recurrence of primary tumor than that of patient 1, who underwent tumor excision and postoperative radiotherapy. Complete tumor excision followed by adjuvant radiotherapy led to successful treatment of recurrent masses. Metastasis was observed in patient 1 at 11 years after radiotherapy. Based on observations they suggest that due to its rare occurrence and atypical clinical findings, orbital ASPS in children is easily misdiagnosed. For achieving long-term disease-free survival in orbital ASPS patients, early diagnosis with multidisciplinary, complete surgical resection combined with adjuvant radiotherapy is essential.