Li-Fraumeni syndrome (LFS) is a rare autosomal dominant disorder caused by a 
mutation in the p53 gene. Melanoma is considered to be a rare, controversial 
component of LFS. The aim of this study is to describe the utility of systematic 
screening for melanoma in patients with LFS and atypical mole syndrome. Two 
28-year-old identical twin sisters with LFS and atypical moles were monitored by 
physical examination, total-body digital photography and dermoscopy be-tween 
2006 and 2014. A total of 117, predominantly dark-brown, reticular naevi were 
identified on case 1 and 105 on case 2. Excisions were performed during the 
evaluation period of 1 in-situ melanoma and 3 basal cell carcinomas in case 1, 
and 1 in-situ melanoma and 1 early invasive melanoma in case 2. The remaining 
melanocytic lesions in both patients were stable during follow-up. The 3 
melanomas were new atypical lesions detected with total-body photography and 
dermoscopy. In conclusion, monitoring LFS patients with total-body photography 
and dermoscopy may be useful to detect early melanoma.