Objective: To report challenges and complications associated with surgical intracranial epidermoid cyst removal from the fourth ventricle of a dog. Study Design: Clinical report. Animal: Labrador retriever (7 years old) with focal seizures. Methods: Magnetic resonance imaging (MRI) revealed an oval lesion in the fourth ventricle. This lesion was heterogeneously hyperintense on T2-weighted and fluid attenuation inversion recovery (FLAIR) images and hypointense on T1-weighted images. Dilatation of the ventricular system was present. A ventriculoperitoneal shunt (VPS) was placed and the fourth ventricle was approached by suboccipital craniectomy. An oval, white mass was identified in the fourth ventricle. Subtotal removal of the mass was performed. Results: On the day after surgery, neurologic deterioration was observed. The dog had severe cerebellar and brainstem dysfunction. Histopathologic examination of the removed tissue revealed an epidermoid cyst. The dog experienced gradual neurologic improvement; however, neurologic deterioration beginning at 6 months resulted in euthanasia 8 months after surgery. On necropsy, epidermoid cyst tissue was identified in the fourth ventricle. Conclusion: Surgical removal of an intracranial epidermoid cyst may be complicated by adhesions between the cyst capsule and surrounding neurovascular structures, causing postoperative morbidity and eventual recurrence of clinical signs. These observations should be taken into account when considering surgical removal of an intracranial epidermoid cyst.