Benign and malignant orbital tumours develop from the orbit or invade it from the surrounding tissues. Ocular melanoma is a rare but potentially devastating malignancy arising from the melanocytes of the uveal tract, conjunctiva, or orbit. The poor overall survival depends mainly on its high metastatic rate. Signs and symptoms are variables mainly depending on the size of the tumour. Its treatment consists generally of surgery, radiotherapy or both. We report a case of a patient suffering from unilateral blindness for the last 10 years with a recent swelling of the orbit. The pathological analysis described a uveal melanoma. The patient benefitted from a total orbital exenteration with reconstruction using a temporal flap. Thereafter, the patient received adjuvant radiotherapy and immunotherapy. The patient was in complete remission. No recurrence was highlighted after 2 years of follow-up.