Oxygen transport, the major function of hemoglobin, is dependent upon reduced heme iron. In the red cell, the heme iron is maintained in the reduced form by the methemoglobin reduction system. When the balance between oxidation and reduction of heme iron is perturbed due to the presence of excessive oxidants, decreased reducing capacity or the presence of abnormal hemoglobin, methemoglobinemia ensues. In most cases methemoglobinemia is transitory and of no major clinical consequence. Occasionally, however, it can be life threatening and must be rapidly diagnosed and treated. When methemoglobinemia is of hereditary nature, either due to deficiency of red cell NADH-methemoglobin reductase or due to the presence of M hemoglobin, it is a lifelong problem. Since most of these patients do not have major disabling symptoms, the treatment is aimed at correction of cyanosis.