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Pigmentary changes in skin can act as an interface to diagnose and monitor various systemic diseases. Endocrine and metabolic disorders can directly or indirectly affect melanin synthesis pathway, thus affecting melanogenesis and skin color. Moreover, these disorders can affect the vascularity of skin and the amount of endogenous nonmelanin pigments that are responsible for skin color. Pigmentary changes can also occur in mucosa, hair, and nails and provide a clue to underlying disease. In this review, we discuss the clinical features, pathogenesis, and treatment of pigmentary changes associated with various endocrine and metabolic disorders. It is imperative for the dermatologists to be aware of these cutaneous manifestations, recognition of which can help in diagnosis and management of underlying condition.