A 2-year-old male entire Cane Corso was presented for investigations into a 1-week history of ambulatory paraparesis and pelvic limb ataxia gradually deteriorating. Magnetic resonance imaging (MRI) revealed intraventricular space-occupying lesions affecting the fourth ventricle and lateral apertures and intradural-extramedullary space-occupying lesions at the level of C7 vertebra, L4-L5, and L7-S1 intervertebral disk spaces. Due to poor quality of life, the patient was euthanized. A post-mortem examination revealed partially encapsulated, multifocally infiltrative, and moderately cellular neoplastic masses. The histological description was similar for all masses. The cells appeared cuboidal with round central nuclei and a moderate amount of eosinophilic cytoplasm and were arranged almost exclusively in single-layered papilliform patterns supported by a fibrovascular stroma. Mitoses were rarely observed (1/2.37 mm2). The primary neoplasm was morphologically most consistent with a choroid plexus papilloma despite drop metastases. This is the first report of a histologically confirmed primary ventricular choroid plexus papilloma causing disseminated MRI-apparent intraventricular and spinal drop metastases.