Polyglandular autoimmune syndrome type II (PGA-II) is a rare immunoendocrinopathy syndrome characterized by the occurrence of autoimmune Addison disease along with diabetes mellitus type 1 and/or autoimmune thyroid disease. Here, we report the case of a 23-year-old female with PGA-II who was followed up at the dermatology and endocrinology clinics of the Universidade Federal do Triangulo Mineiro, located in the state of Minas Gerais, Brazil. First, the patient presented diffuse skin hyperpigmentation, vitiligo; and in sequence, due to vomiting, appetite and weight loss, hypoglycemia, amenorrhea, and galactorrhea, the patient was then diagnosed with PGA-II. The patient also presented intense hyperprolactinemia due to primary hypothyroidism. The late diagnosis of PGA-II is frequent because the disorder is uncommon and has non-specific clinical manifestations. This report emphasizes the significance of a timely diagnosis and appropriate treatment to reduce morbidity and mortality associated with these diseases, especially Addison disease. The present study reports a rare case of a patient with PGA-II with primary amenorrhea associated with hyperprolactinemia. Resumo A sindrome poliglandular autoimune tipo 2 (SPGA-2) e uma sindrome de imunoendocrinopatia rara caracterizada por doenca de Addison autoimune associada a diabetes mellitus tipo 1 e/ou doencas tireoidianas autoimunes. Relatamos aqui o caso de uma paciente de 23 anos de idade com SPGA-2 que foi acompanhada nos ambulatorios de dermatologia e endocrinologia da Universidade Federal do Triangulo Mineiro, localizada no estado de Minas Gerais, Brasil. Primeiramente, a paciente apresentou hiperpigmentacAo cutanea difusa e vitiligo; posteriormente, por apresentar vomitos, hiporexia, perda ponderal, hipoglicemia, amenorreia e galactorreia, foi diagnosticada com SPGA-2. A paciente apresentou tambem intensa hiperprolactinemia secundaria apenas ao hipotireoidismo primario. E comum o diagnostico tardio da SPGA-2, pois a doenca e rara e apresenta manifestacoes clinicas inespecificas. Este relato de caso enfatiza a importancia do diagnostico e tratamento precoces com o objetivo de reduzir a morbimortalidade associada a essas doencas, especialmente a doenca de Addison. O presente estudo descreve um caso raro de uma paciente com SPGA-2 com amenorreia primaria associada a hiperprolactinemia.