Neurothekeomas are uncommon benign superficial cutaneous tumors with various histologic patterns, such as myxoid, cellular, or mixed types, depending primarily on the quantity of myxoid matrix present. They generally affect the head and neck, are more common in women than men, and typically start in the second and early third decades of life. Once believed to originate from the nerve sheath, the source is now thought to be fibroblasts that can differentiate into myofibroblasts and entice histiocytes. Neurothekeomas can present a significant challenge for pathologists to diagnose, and strict histomorphologic features and immunohistochemistry studies are essential to establish the diagnosis. We present a recurrent scalp myxoid Neurothekeoma case in a 7-year-old girl and review the pertinent literature.