Malignant melanoma of oral cavity (MMOC) is rare and aggressive condition, representing 0.5% of all oral malignant neoplasms. The hard palate and maxillary alveolar mucosa are the most frequently sites affected. This case reports an MMOC in a 19-year-old woman who presented a painless dark lesion in the upper lip. Biopsy was performed, and the histologic findings were of chronic inflammation (2008). Four years later, the patient presented a pigmented nodule lesion in the upper lip mucosa and maxillary alveolar mucosa. A new biopsy was performed, and the diagnosis of MMOC was established with complaints of lymph nodes metastasis. The patient was submitted to surgery, chemotherapy, and radiotherapy. Two years after treatment, the patient presented lung metastasis and was submitted to partial lung resection. This case emphasizes the importance of the follow-up of black lesions and the early diagnosis for a better prognosis in aggressive lesions such as MMOC. Malignant melanoma of oral cavity (MMOC) is rare and aggressive condition, representing 0.5% of all oral malignant neoplasms. The hard palate and maxillary alveolar mucosa are the most frequently sites affected. This case reports an MMOC in a 19-year-old woman who presented a painless dark lesion in the upper lip. Biopsy was performed, and the histologic findings were of chronic inflammation (2008). Four years later, the patient presented a pigmented nodule lesion in the upper lip mucosa and maxillary alveolar mucosa. A new biopsy was performed, and the diagnosis of MMOC was established with complaints of lymph nodes metastasis. The patient was submitted to surgery, chemotherapy, and radiotherapy. Two years after treatment, the patient presented lung metastasis and was submitted to partial lung resection. This case emphasizes the importance of the follow-up of black lesions and the early diagnosis for a better prognosis in aggressive lesions such as MMOC.