Protection centers on near-total UV avoidance and early cancer surveillance:

*UV avoidance: Strict indoor lifestyle during daylight hours; window films that block UV-A/UV-B on homes, cars, and schools; UV-protective clothing (UPF 50+), wide-brimmed hats, and UV-blocking face shields/visors when outdoors is unavoidable.
*Broad-spectrum sunscreen: SPF 50+ with both UVA and UVB coverage, reapplied every 2 hours during any outdoor exposure.
*Eye protection: Wraparound UV-blocking sunglasses, as xeroderma pigmentosum (XP) also causes corneal and conjunctival damage, photophobia, and ocular tumors.
*Vitamin D monitoring: Since sun avoidance is near-total, patients need periodic vitamin D level checks and supplementation.
*Skin surveillance: Dermatologic exams every 3–6 months for early detection of basal cell carcinoma, squamous cell carcinoma (SCC), and melanoma, which can appear by age 10 in untreated patients.
*Neurological monitoring: Annual neurology and audiology assessment in XP subtypes with neurodegeneration risk (notably XP-A, XP-D).
*Genetic counseling: For parents planning future pregnancies, given the autosomal recessive inheritance and 25% recurrence risk per pregnancy.

With rigorous photoprotection instituted early, studies show skin cancer incidence in XP patients can be reduced substantially compared to those without strict UV avoidance, early diagnosis and behavior change are the single biggest determinants of outcome.