Background: The lymphoma is the most common childhood malignancy in the head and neck. Approximately 15% of head and neck lymphomas in children affect the Waldeyer's ring. Early diagnosis and treatment are of great importance in the prognosis of tonsillar lymphoma patients. Objectives: To realize a systematic review of the literature on the clinical manifestations present at diagnosis of tonsillar lymphoma in pediatric patients. Date Source: Articles in English, Spanish or Portuguese in the last 15 years about lymphoma in palatine tonsil in children from PubMed/Medline, LILACS, IBECS, Cochrane, SCIELO, BIREME and Scopus. Study eligibility criteria: It was included articles and case reports that covered the pediatric age group, up to 18 years old and that contained information of the clinical manifestations of tonsillar lymphoma at diagnosis. Results: We found 87 articles of which 18 were included; there were 66 cases of lymphoma of palatine tonsils. The most common clinical manifestations found in children with lymphoma in palatine tonsils were unilateral tonsillar enlargement (72.7%), alteration in appearance of the tonsil (45.4%) and cervical lymphadenopathy (30.3%). The presence of B symptoms occurred in only 16% of the patients. Burkitt was the most common type of lymphoma found. Conclusion: The most common clinical manifestations of lymphoma in palatine tonsil are the tonsils asymmetry, alteration in the appearance of the mucous and cervical lymphadenopathy. 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