Usually, PLM presents as a solitary lesion, but approximately 5% of patients have multiple PLMs, usually in association with complex genetic syndromes such as myotonic dystrophy, Gardner syndrome, Turner syndrome, Rubinstein-Taybi syndrome, Goldenhar syndrome, Kabuki syndrome, Sotos syndrome, trisomy 9, gliomatosis cerebri, panhypopituitarism, MYH-associated polyposis and DICER1 syndrome [1]. This evidence concerns the gene MUTYH and DICER1-related tumor predisposition.