Background and Importance: An association with multiple primary intracranial tumors of different histologic types not relating to radiotherapy or phacomatosis is a rare event. To the best of the authors knowledge, a giant osteoma originating from the inner surface of the petrous bone associated with an ipsilateral clivus meningioma has never been reported. Clinical Presentation: A 43-year-old patient with trigeminal neuralgia was found to have a posterior fossa bony lesion measuring 4 cm, with a solid enhancing lesion in juxtaposition and a separate bony lesion on the occipital bone on magnetic resonance and computed tomography imaging. A satisfactory resection of the coexisting tumors in the petroclival region was performed through a subtemporal transtentorial approach. The tumors were finally confirmed as an osteoma and a meningioma by pathologic evaluation. The patient demonstrated an improvement in her trigeminal neuralgia over a 3-month postoperative period of time. Conclusions: This case presents new clinical and radiographic findings associated with a rare lesion. Dependent tumors that one tumor may act as an irritating agent for the local proliferation of the other most likely accounted for this pattern of tumoral linkage. A satisfactory surgical result was achieved through a relatively minimal approach and different strategies. Trigeminal neuralgia, the initial presenting symptom, improved during the follow-up period. © 2013 Wolters Kluwer Health, Inc. All rights reserved.