Dyschromatosis symmetrica hereditaria (DSH) is a pigmented genetic skin disorder with an incompletely understood pathogenesis characterized by reticular hyper- and hypopigmented skin patches on the dorsal aspect of the extremities, freckle-like patches on the face, and unaffected palms and feet. There is no effective treatment available. Glucose-6-phosphate dehydrogenase (G6PD) deficiency has not been reported in the literature of DSH. We describe for the first time a case of DSH with G6PD deficiency and a family history of psychosis.