A 66-year-old male patient was admitted due to high fever, severe headaches and disturbance of consciousness. Meningitis was confirmed by lumbar puncture and intravenous antimicrobial therapy was started. Since he had undergone radical tympanomastoidectomy 15 years before, otogenic meningitis was suspected, so the patient was referred to our department. Clinically, the patient manifested watery discharge from the right nostril. Microbiological analysis verified Staphylococcus aureus in a cerebrospinal fluid (CSF) sample acquired by lumbar puncture. Radiological work-up, including computed tomography and magnetic resonance imaging scans, showed an expanding lesion of the petrous apex of the right temporal bone disrupting the posterior bony wall of the right sphenoid sinus with radiological characteristics indicating cholesteatoma. Those findings confirmed rhinogenic meningitis caused by expansion of the petrous apex congenital cholesteatoma into the sphenoid sinus, allowing nasal bacteria to enter the cranial cavity. The cholesteatoma was removed completely by the simultaneous transotic and transsphenoidal approach. Since the right labyrinth was already non-functional, there was no surgical morbidity after labyrinthectomy. The facial nerve remained preserved and intact. The transsphenoidal approach enabled removal of the sphenoid portion of the cholesteatoma and two surgeons met together at the level of the retrocarotid segment of the cholesteatoma, completely removing the lesion. This case represents an extremely rare condition in which a petrous apex congenital cholesteatoma expanded through the petrous apex to the sphenoid sinus, causing CSF rhinorrhea and rhinogenic meningitis. According to available literature, this is the first case of petrous apex congenital cholesteatoma causing rhinogenic meningitis successfully treated with the simultaneous transotic and transsphenoidal approach.