Pseudotumor cerebri syndrome (PTCS) is an uncommon disease in children. On-time diagnosis and treatment can prevent irreversible visual loss. Although headache is the most common complaint of children with this syndrome, the present case report reported a child with neck rigidity and torticollis, declined by the reduction of intracranial pressure. Despite the importance of torticollis and neck rigidity presented in various significant neurological disorders in need of thorough investigations, in the case of unexplained symptoms of those disorders, it is recommended to consider fundoscopic examinations for PTCS to prevent its vital complications.