Introduction Primary cerebellopontine angle melanoma (PCPAM) is one of the most rare primitive brain tumors. Its management and prognosis are drawn from very few published cases. Aim The aim of this study was to provide an approach to help practitioners diagnosing similar cases. Material and methods A systematic review was conducted. A PCPAM case is also presented. Results The search yielded 13 cases of PCPAM (53% were males) with a median age of 32 years (range 26–56 years). The most frequent symptoms were hearing loss (77%) and ataxia (62%). All underwent neurosurgical removal of the tumor due to worsening of symptoms. Post-surgery follow-up showed that only half of the cases had free-of-disease survival beyond a year. The other half had a poor outcome few months after surgery. We present a 77-years-old female patient with PCPAM with clinical characteristics different from those described in the literature. Discussion Results indicate that diagnosis criteria are still lacking specificity. Therefore, clinical features have led clinicians to reconsider the diagnosis more than once. New techniques, such as PET-scan and genetic analysis can greatly assist in the search for the primary tumor. Nowadays, complete resection and radiotherapy are still the gold standard treatment. Prognosis differs between the cases, but age and tumor biology are the main indicators of survival. Conclusions We strongly suggest strengthening the surveillance of patients with PCPAM as the management and prognosis differ significantly from those with metastatic melanoma.