We present the case of a 46-year-old woman diagnosed with a primary oesophageal melanoma (PEM), who was treated with radical surgery followed by combined chemoimmunotherapy (interferon, carboplatin, dacarbazine and external radiotherapy) and who achieved a complete response after this treatment. PEMs are rare malignancies, with less than 300 cases described in the literature. The main differential diagnosis is with metastases of skin or ocular malignant melanomas. They are usually diagnosed at advanced stages and prognosis is typically poor. The main treatment modality should be radical surgery. The role of adjuvant treatment is uncertain, although some long responses have been seen with the use of chemotherapy or immunotherapy alongside surgery.