Pseudohypoaldosteronism is a rare syndrome occurring during early infancy, which is mainly characterized by salt-depletion crises. Sodium chloride is lost via the kidneys resulting in a reduced sodium level and raised potassium level in the serum, leading to life-threatening disturbances of water and acid-base concentration. The excessive sodium loss seems to be caused by an unresponsiveness of the renal tubules to endogenous and exogenous mineralocorticoids. The colon, salivary and sweat glands, which are also involved in sodium reabsorption, may likewise be affected by the disease. We describe skin changes in two siblings suffering from pseudohypoaldosteronism, who developed seborrheic dermatitis, folliculitis or miliaria rubra-like lesions during salt-depletion crises. Biochemical analysis revealed a highly increased sodium-chloride concentration in the sweat, saliva, urine and stool of both patients. Destructive inflammatory reactions could be demonstrated histologically within and around the dermal sweat glands, thus indicating the important role of the sweat system in the pathogenesis of skin lesions in pseudohypoaldosteronism.