Conjunctival malignant melanoma is a rare tumor with a high risk of local recurrence, lymph node and systemic metastases. The aim of this study was to correlate tumor thickness, tumor ulceration, high mitotic rate, epithelioid cells with the presence of metastases and death from conjunctival malignant melanoma. We report the case of a 33-year-old patient who presented with a left eyelid ptosis associated with an eyelid prominence, foreign body sensation in the eye, and bloody discharge, symptoms occurring about one month earlier. Ophthalmologic examination revealed in eyelid conjunctiva two vegetant and ulcerative tumors of 8/6 mm and 3/3 mm. The two tumors were surgically removed with safety margins. The diagnosis of amelanotic malignant melanoma of the conjunctiva with brain metastasis was made by routine morphological methods and immunohistochemical reactions (HMB45, vimentin, S100 protein). Systemic metastases (skin, brain, lung, liver, kidney, peritoneal) and peripancreatic lymph node metastases were detected at 1.9 years after the diagnosis of conjunctival malignant melanoma. The patient died three months after the surgical excision of brain metastasis. Early diagnosis is essential to prevent tumor recurrence, ocular invasion, systemic and lymph node metastases, and preserving visual function.