Objective: The majority of ocular adnexal lymphomas are marginal zone lymphomas, which occur rarely in children. This case report describes a 6 years old child with a precursor B lymphoblastic lymphoma presenting in the ocular adnexa. The combination of multi-agent chemotherapy with adjuvant radiotherapy seems to be necessary in order to achieve a complete remission of this subtype of lymphoma's in ocular adnexa. Design: Retrospective case study. Method: A review of the clinical, pathological, radiological findings and follow-up in a patient from the files available at our center, which were reviewed between the years 1974 and 2004.