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Abstract Objective To evaluate the incidence of tonsil and adenoid malignancy in pediatric patients in Israel compared to the known literature and to revisit the common practice of routine histopathologic examination of tonsils and adenoids. Methods Analysis of the Israel National Cancer Registry data on pediatric tonsil and adenoid malignancies between the years 2005 and 2015, and a systematic literature review of all relevant articles that reported on malignancies amongst pediatric patients who underwent tonsillectomy with or without adenoidectomy. Results Only seven cases of tonsillar malignancies were documented out of 152,352 (0.0052%) surgeries in the pediatric population. All malignancies were lymphoproliferative and mainly Burkitt's lymphoma. In our medical center no malignancy was found in 2165 patients in the same age groups and time period. We found the incidence of tonsillar malignancy in Israel to be significantly lower (p = 0.013) compared to the previously described literature. Conclusion The incidence of malignancy in routine histopathological examination of tonsils and adenoids is Israel is very low when compared to the known literature. According to these results, we suggest that routine histopathological examination of all such samples is not clinically justified. [ABSTRACT FROM AUTHOR]