There are many types of neoplasms in or around the foramen of Luschka (FL), and definitive diagnosis in some cases requires knowledge of imaging findings. The uncommon and challenging neoplasms with FL involvement considered in this study are exophytic brainstem glioma, primary glioblastoma of the cerebellopontine angle (CPA), primary anaplastic ependymoma of the CPA, choroid plexus papilloma of the FL, solitary FL choroid plexus metastasis, extraskeletal myxoid chondrosarcoma of the jugular foramen, paraganglioma of the jugular foramen, exostosis of the jugular foramen, psammomatous meningioma in the lateral cerebellar medullary cistern, epidermoid tumor of the fourth ventricle, and a hypoglossal schwannoma. These neoplasms may have overlapping clinical and imaging features, but some have relatively distinct imaging features. Knowledge of the key clinical and magnetic resonance imaging features of these unusual lesions with FL involvement is important for radiologists to improve diagnostic ability and to assist the referring physician in the appropriate management of the patient.