Simple SummaryChoristomas are rare malformations consisting of normal mature tissue in an abnormal location. Neuroglial choristomas consist of heterotopic mature neural tissue and, in human medicine, they are predominantly reported in the head and in the neck, except for one recent case reported in a foot of a child. In domestic animals, neuroglial choristomas are exceedingly rare, reported only in the retina of a dog, in the pharynx and in the skin of two kittens, and within the oropharynx of a harbor seal. In this report we describe the first ovarian case of neuroglial choristoma in canine species. The lesion was present in a three-year-old intact female Jack Russell Terrier that, seven years after ovariectomy, is still clinically normal. Together with a recent case described in the foot of a child, this case confirms that neuroglial choristoma may also be found far from the skull or spine, supporting the hypothesis that they may arise from an early embryological migration defect.Neuroglial choristomas are rare malformations consisting of heterotopic mature neural tissue at a site isolated from the brain or spinal cord. In human medicine, neuroglial choristomas are predominantly reported in the head and in the neck, except for one recent case reported in a foot of a child. In domestic animals, neuroglial choristomas are exceedingly rare, reported only in the retina of a dog, in the pharynx and in the skin of two kittens, and within the oropharynx of a harbor seal. A three-year-old intact female Jack Russell Terrier presented for elective ovariectomy exhibited a cystic lesion 2 cm in diameter expanding in the right ovary. Histological examination of the lesion revealed a mass composed of well-organized neuroglial tissue. Immunohistochemistry with primary antibodies against GFAP, NSE, and IBA-1 confirmed the neuroglial origin of the mass. At the time of this writing, 7 years after ovariectomy, the dog was clinically normal. Together with a recent case described in the foot of a child, this case confirms that neuroglial choristoma may also be found far from the skull or spine, supporting the hypothesis that they may arise from an early embryological migration defect.