Neurothekeomas (nerve sheath myxomas) are uncommon benign tumors of nerve sheath origin. We describe an infant with a neurothekeoma and review the literature of neurothekeomas in children and adults. Neurothekeomas have been reported in 292 patients whose ages have ranged from 15 months to 84 years. They occur twice as often in women as in men. Neurothekeomas were most commonly located on the upper extremities and the head and neck. They also occurred on the trunk, the lower extremities, and mucosa. Histologic variants of neurothekeomas include classical, cellular, and mixed tumors. Surgical excision was the most common treatment. Recurrences were attributed to an incomplete excision; treatment of these tumors is by complete excision. Neurothekeomas should be included in the differential diagnosis of dermal nodules in infants and children.