Craniopharyngiomas are benign brain tumors that can often be cured surgically. A small fraction of unresectable tumors can progress and cause significant morbidity and even death. Unfortunately, WNT activated tumors lack clinically-validated targeted therapies in the pediatric population. Herein, we describe a patient with a multiply recurrent adamantinomatous craniopharyngioma with WNT activation. We utilized the MEK inhibitor binimetinib with noted interval decrease in tumor size. This demonstrates the possible utility of MEK inhibitors in WNT activated craniopharyngiomas.