The authors describe a clinical case of a 5-year-old girl with history of thickening and yellow discoloration of the great toenails with lateral deviation, since her first month of life. The patient was diagnosed with congenital malalignment of the great toenails. The congenital malalignment of the great toenails was first described as a nosological entity in 1983, by Baran et al. It is a nail disorder in which the nail plates are laterally deviated with respect to the longitudinal axis of the distal phalanx. Other ad­ditional clinical features include yellow-green discoloration, trans­verse ridging (Beau lines) and thickening with dystrophy.