While the effectiveness of MEK inhibitors such as selumetinib is established in <i>NF1</i>-associated inoperable plexiform neurofibromas, their use in managing hyperactive <i>KRAS</i>-driven epidermal nevi and hypertrophic neuropathy remains unproven, and this case, to our knowledge, is the first such case to be reported. Here, KRAS is linked to plexiform neurofibroma.