A 22-month-old girl presented with neck pain and stiffness and magnetic resonance imaging showed an extradural mass extending from C2 through the C4 level with moderate to severe compression of the cord. A left unilateral C2-C4 laminectomy was performed revealing an extradural rubbery tumor; a small biopsy was obtained. Examination of stained tissue revealed the presence of a parasitic worm that was identified as a gravid female Onchocerca lupi. A magnetic resonance imaging at 7 weeks follow-up showed a significantly decreased size of the enhancing lesion and the patient's symptoms gradually resolved. This is the first report of zoonotic O. lupi in the United States. The parasite has been reported in dogs and cats in the western United States, and from people in four cases reported from Europe. A great deal more needs to be learned, including full host range and geographic distribution, before we fully understand O. lupi infections in animals and man.