Background: Ovarian infiltration in pediatric non-Hodgkin lymphoma (NHL) at presentation is rare and information on outcome is scarce and mainly based on case reports and small series. Procedure: Evaluation of clinical characteristics and outcome of ovarian infiltrated pediatric NHL cases of a single center, and an extensive review of the all cases reported so far in literature. Results: At presentation, 6/60 female NHL cases of our center had ovarian infiltration, and combining these cases with earlier case reports, a total of 42 cases were identified. Median age at presentation was 10.9 years (range 0-18), and all but one had a B-cell immunophenotype, with 32/42 cases being classified as Burkitt. Bilateral involvement was reported in 26/41 cases, of which 22 were bilaterally ovariectomized as first treatment. All cases were treated with chemotherapy. Relapses were reported in 9/36 and death in 16/36. After follow-up in our center (median 13.4 years), in 2 cases anti-Müllerian hormone (AMH) values were available (2.1 and 0.9μg/L), in non-ovarian cases median 2.2μg/L. Conclusions: We conclude that in case of ovarian tumors with negative markers, NHL should be considered in order to avoid unnecessary surgery. Pediatr Blood Cancer 2013;60:2054-2059.