| Summary: | Abstract Xeroderma pigmentosum (XP) is a rare genetic disease, which vital prognosis is conditioned by the occurrence of cancers essentially of the skin and ocular surfaces, requiring an early and adapted management. Radiation therapy (RT) is a very effective modality in the therapeutic arsenal alongside surgery, but it remains underused as it is wrongly considered to be deleterious for these patients. In this article, we report the case of a 10‐years‐old girl with XP treated with external beam RT for a squamous cell carcinoma (SCC) of the right ocular conjunctiva. The clinical tolerance was excellent and we obtained a good tumoral response. Therefore, the place of RT in these patients could/should be reconsidered, especially since these suspicions have still not been confirmed.
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