Toxocariasis caused by Toxocara canis or less frequently by T. catis is a common parasitic infection worldwide. Clinical spectrum in humans can vary from asymptomatic infection to serious organ disfunction depending on the load of parasite, migration target of the larva and the inflammatory response of the host. Transverse myelitis (TM) due to toxocariasis is an uncommon illness identified mainly as case reports in literature. In this report, a case of TM who was diagnosed as neurotoxocariasis by serological findings has been presented. A 44-year-old male patient complained with backache was diagnosed as TM in a medical center in which he has admitted two years ago, and treated with pregabalin and nonsteroidal drugs for six months. Because of the progression of the lesions he readmitted to another center and treated with high dose steroid therapy for three months. After six months of follow up, improvement has been achieved, however, since his symptoms reccurred in the following year he was admitted to our hospital. Magnetic resonance imaging (MRI) examination revealed a TM in a lower segment of spinal cord. He was suffering with weakness and numbness in the left lower extremity. There was no history of rural life or contact with cats or dogs in his anamnesis. Physical examination revealed normal cranial nerve functions, sensory and motor functions. There has been no pathological reflexes, and deep tendon reflexes were also normal. Laboratory findings yielded normal hemogram and biochemical tests, negative PPD and parasitological examination of stool were negative for cysts and ova. Viral hepatitis markers, anti-HIV, toxoplasma-IgM, CMV-IgM, rubella-IgM, EBV-VCA-IgM, VDRL, Brucella tube agglutination, echinococcus antibody, autoantibody tests and neuromyelitis optica test were negative. Examination of CSF showed 20 cells/mm(3) (mononuclear cells), 45 mg/dl protein and normal levels of glucose and chlorine. In both serum and CSF samples of the patient Toxocara-IgG antibodies were detected by Western blot (WB) assay. Low molecular weight bands (30-40 kDa) were detected in both of the samples by repeated WB testing. CSF revealed more intense bands suggesting local antibody production. Therefore the patient was diagnosed as neurotoxocariasis, and treated with steroid and mebendazole for six weeks. Clinical improvement was detected in the case and thoracic MRI revealed significant improvement in myelitis signs two months after treatment. In conclusion, toxocariasis should be considered in the differential diagnosis of TM although the involvement of central nervous system is rare and serological testing should be performed properly in the serum and CSF samples for the diagnosis.