Objective: To present a rare case of a primary malignant melanoma of the central nervous system presenting as a cerebellopontine angle (CPA) tumor and to delineate aspects of the clinical presentation and magnetic resonance imaging scan characteristics that may suggest this unusual condition.
Clinical presentation: The clinical presentation consisted of a short duration of right-sided sensorineural hearing loss, facial weakness, and ataxia in a previously healthy man. Brain magnetic resonance imaging scans showed a right-sided CPA tumor exhibiting shortening of T1 and T2 relaxation times, but overall neuroradiological features were not consistent with any commonly occurring CPA tumors.
Intervention: Gross total excision of the lesion was accomplished via a right suboccipital craniectomy. Histological examination revealed a malignant melanoma. A detailed search excluded extracranial primary melanoma.
Conclusion: Correlation of clinical and imaging findings offer the most important clues in the diagnosis of such unusual primary malignant tumors of the CPA. A history of rapid onset of audiovestibular symptoms, presence of facial palsy, and shortening of T1 and T2 relaxation times on magnetic resonance imaging scans should arouse the clinician's suspicions.