A 9-year-old boy with pseudotumor cerebri who presented with neck pain and an accompanying torticollis is described. The patient exhibited bony abnormalities of the upper cervical spine and facial asymmetry that suggested a congenital torticollis. Reduction of the increased cerebrospinal fluid pressure by lumbar puncture resulted in a prompt and dramatic resolution of the cervical symptoms and signs. Increased cerebrospinal fluid pressure should be added to the list of those disorders that may give rise to reversible torticollis.