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Autoimmune polyendocrine syndrome (APS) is a constellation of multiple endocrine and various autoimmune diseases. The hallmark features of APS are gradual onset, circulating autoantibodies, and tissue or organ infiltration by lymphocytes. There are genetic basis and failure of the immune system to maintain self-tolerance to a variety of molecules, which manifest as autoimmunity over a period of time. Age of onset of the syndrome may range from early infancy to adulthood, new onset of autoimmunity of the given syndrome can manifest thoughout life. We report a case of an adolescent female with endocrine and non-endocrine manifestation of APS, starting at a very young age of 7 years with nephritis and hypertension as an unusual association.

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