Objective and Importance: The authors report the first known case of a giant multiloculated grape-like enterogenous cyst extending from the upper cervical canal to the ambient cistern. Clinical Presentation/Methods: We report the case of a 40-year-old male who had a prior transmastoid craniotomy at an outside facility 14 months prior with an indeterminate diagnosis, who presented to the University of New Mexico with recurrent headaches and nausea. Scans demonstrated a giant multiloculated cystic lesion in the right cerebellopontine angle that extended superiorly and inferiorly with brainstem compression and hydrocephalus. Intervention/Results: We took the patient to the operating room for a retrosigmoid suboccipital craniectomy for tumor resection. Post-operatively, the patient improved but required ventriculoperitoneal shunting for continued communicating hydrocephalus. Conclusion: This is the first known case of a giant multiloculated grape-like enterogenous cyst extending simultaneously from the upper cervical canal to the ambient cistern. Enterogenous cysts should be considered on the differential diagnosis of giant grape-like lesions extending from the cervical canal to the prepontine cistern.