Abstract: | Glandular schwannoma is an infrequent tumor, especially in the pediatric age group. We report a case in which the tumor developed in the retroperitoneum of a 17-month-old girl without stigmata of Von Recklinghausen's disease (VRD). She presented a local recurrence and bone and pulmonary metastases at 11, 23, and 30 months, respectively, after tumor resection. The pathologic findings in the original tumor did not permit us to predict its potential to metastasize. The ultrastructural characteristics of both glandular and schwannian elements are described. The rarity of this neoplasia in children and the absence of histological parameters of aggressiveness are emphasized. |