Primary meningeal melanocytoma is a rare benign tumor in the central nervous system (CNS), comprising less than 0.1% of all intracranial tumors. A 44-year-old man presented with occipital headache, nausea, and vomiting. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a well-defined intradural extramedullary mass lesion at the craniovertebral junction (CVJ). Gross total removal was achieved, and the patient improved symptomatically. The pathologic findings were consistent with meningeal melanocytoma. No tumor recurrence was seen on follow-up MRI two years after surgery. Cases of primary meningeal melanocytoma located at the CVJ are rare. The preoperative differential diagnosis of meningeal melanocytoma from meningioma is sometimes difficult because of their similar appearance on CT and MRI. Complete surgical removal is curative for most cases. We present a case of gross total removal of a meningeal melanocytoma located in the CVJ with references to the literature.