Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy is also known as autoimmune polyglandular syndrome type I (APS-I). We report a case of late presented APS-I with non-endocrine manifestations. A 41-year-old male presented with chronic mucocutaneous candidiasis as the first manifestation of the disease, followed by adrenal insufficiency. Other associated diseases that developed in time included gonadal insufficiency, alopecia, vitiligo, chronic urticaria. chronic active hepatitis and malabsorption. Although our patient presented late with APS-I and non-endocrine manifestations, APS-I usually occurs in childhood and is an autosomal recessive disorder characterized by a combination of autoimmune manifestations affecting endocrine and non-endocrine organs. In conclusion, patients with autoimmune endocrine disease should be followed on a regular basis.