Hydrocephalus is one of the most common central nervous system malformations in domestic dogs, yet they are poorly documented and studied in wild carnivoran mammals. A pup of raccoon dog (Nyctereutes procyonoides) was rescued and brought to Wildlife Center. The pup showed generalized ataxia, a domed skull, and an open bregmatic fontanelle. Ultrasound and MRI showed severe enlargement of the lateral ventricle with the loss of septum pellucidum resulting in a single large ventricle and cervical syringohydromyelia. Although treatment was attempted, the animal was euthanized due to poor prognosis. At necropsy, macroscopic findings were identical to the diagnostic imaging, where marked enlargement of the calvarium, and attenuated gyri and sulci were observed. Finally, hydrocephalus was confirmed. Here, we describe a case of hydrocephalus in a raccoon dog (Nyctereutes procyonoides).