Cysticercosis is the most common parasitic infection of the cerebral parenchyma and spinal cord involvement is rare. In spinal cord, it affects more commonly thoracic spinal cord while cervical involvement is rare. We report the case of a 30-year-old female patient with isolated cervical intramedullary cysticercosis. Patient presented with pain in neck and right upper limb. Her magnetic resonance imaging (MRI) cervical spine revealed dumbbell-shaped intramedullary lesion at C3 to C4 level. Patient underwent surgery with complete excision of cervical intramedullary lesion. Histopathological report confirmed cysticercosis. Postoperatively, patient had complete relief in her clinical symptoms. In this report, we also discuss the principles of diagnosis and treatment of intramedullary cysticercosis in combination with literature review. Spinal neurocysticercosis should be considered as a differential diagnosis of spinal mass lesion in patients residing in endemic area, such as India. Both surgical therapy and medical therapy have a role in the management of spinal cysticercosis.