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Primary melanocytic tumors of the central nervous system (CNS) are rare lesions, but primary sellar tumors are rarer.Only 10 cases have been reported, and they are often misdiagnosed as pituitary macroadenoma.We report the case.of a 54-year-old Chinese man who developed progressive bitemporal hemianopsia and visual loss.Magnetic resonance image (MRI) revealed a giant intrasellar and suprasellar clouded lesion ad hering to the optic chiasm, hypothalamus, and hypophyseal stalk that was suspected of being a hemorrhagic pituitary macroadenoma.Because of the atypically giant, hemor rhagic, and upward-growing lesion, an initial trans-sphenoidal approach failed, and subsequent transfrontal craniotomy was adopted to achieve macroscopically complete resection.Histopathological findings revealed a benign melanocytic tumor.Despite an extensive search, no other primary or secondary site was found.Considering the rela tively benign lesion, effective surgery, and severe postradiotherapy reaction, the patient received no further treatment and is still alive at the 7-year follow-up.Primary sellar melanocytic tumors are exceptional lesions that are difficult to diagnose before operat ing and/or obtaining pathological findings.The pathological classification and extent of surgical resection may play a key role in the prognosis.Once this type of lesion is suspected, the transfrontal approach may achieve preferable exposure and resection.Complete surgical resection may be sufficient for relatively benign lesions; otherwise,stereotactic fractionated radiotherapy is indicated.More cases should be reported to improve the treatment strategy.