BackgroundPrimary melanoma of the adrenal gland is exceptionally rare as demonstrated by the few cases reported in the medical literature, and it has a high fatality rate. We present the case of a patient with two relapses and survival to date.Case reportWe describe the case of a 58-year-old Caucasian woman who consulted her doctor with symptoms of asthenia, anorexia and weight loss. A mass was palpated in her abdomen at the height of the left hypochondrium. A computed tomographic scan revealed a retroperitoneal mass measuring 10 cm × 15 cm originating in the left adrenal gland. A left nephroadrenalectomy and splenectomy were performed. Histopathologically, the retroperitoneal mass corresponded to a melanoma, and no primary melanoma was found in any other location. The patient was treated with interferon-α-2b. Three years after her diagnosis the patient presented with a retroperitoneal relapse of the mass measuring 7.2 cm, which was removed. Five years after the first relapse a new retroperitoneal relapse mass was diagnosed, which was also removed. Since then the patient has been healthy and free from illness.ConclusionHistological and immunohistochemical studies, together with the criteria described by Ainsworth et al. and Carstens et al., allowed us to diagnose primary melanoma of the adrenal gland.