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It is characterized by cutaneous and uterine leiomyomas and an increased risk of developing renal cell carcinoma (RCC), which is usually adult-onset. HLRCC-related RCC tends to be aggressive and can metastasize even when the primary tumor is small. Data on children and adolescents are scarce. Herein, we report two patients from unrelated Dutch families, with HLRCC-related RCC at the ages of 15 and 18\u00a0years, and a third patient with an <jats:italic>FH<\/jats:italic> mutation and complex renal cysts at the age of 13. Both RCC\u2019s were localized and successfully resected, and careful MRI surveillance was initiated to monitor the renal cysts. One of the patients with RCC subsequently developed an ovarian Leydig cell tumor. A review of the literature identified 10 previously reported cases of HLRCC-related RCC in patients aged younger than 20\u00a0years, five of them presenting with metastatic disease. These data emphasize the importance of recognizing HLRCC in young patients to enable early detection of RCC, albeit rare. They support the recommendations from the 2014 consensus guideline, in which genetic testing for <jats:italic>FH<\/jats:italic> mutations, and renal MRI surveillance, is advised for HLRCC family members from the age of 8\u201310\u00a0years onwards.<\/jats:p>","DOI":"10.1007\/s10689-019-00155-3","type":"journal-article","created":{"date-parts":[[2019,12,2]],"date-time":"2019-12-02T11:30:12Z","timestamp":1575286212000},"page":"55-63","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":43,"title":["Renal cell carcinoma in young FH mutation carriers: case series and review of the literature"],"prefix":"10.1007","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3156-5229","authenticated-orcid":false,"given":"J. 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