Malignant peripheral nerve sheath tumors were detected in three of the 38 patients with NF1 whole gene deletions within the observed time period (type-1 group: n = 2; atypical group: n = 1), resulting in a prevalence of 7.9% (type-1 group: 7.4%; atypical group 9.1%), respectively. Here, NF1 is linked to malignant peripheral nerve sheath tumor.