A comprehensive retrospective study revealed that complete tumor removal was successful in only 15% of cases, while 43% of patients who underwent surgery experienced PN recurrence.13) In 2020, selumetinib, an oral selective MEK1/2 inhibitor, was approved by the United States Food and Drug Administration for the treatment of NF-1-associated symptomatic inoperable PNs in children older than 2 years.14) Han et al.15) systematically evaluated the efficacy and safety of selumetinib in patients with NF-1. Here, MAP2K1 is linked to poikiloderma with neutropenia.