Congenital hyperpigmentation in the acro-genital area and simultaneous occurrence of ganglioneuroma in the esophagus have yet to be reported. Herein, we report a 4-month-old girl presenting with feeding difficulty by esophageal ganglioneuroma and symmetrically distributed brown pigmented patches on the lips, axillae, dorsa of fingers and toes, and genital area. Although the esophageal stricture was resolved by surgical removal of ganglioneuroma, her skin manifestations remained for over 2 years.