Congenital malalignment of the great toenail is an under-estimated dystrophic disorder of unknown origin characterized by lateral deviation of the nail plates, which are not parallel to the major axis of the distal phalanx. It usually presents in infancy or childhood, while late onset is uncommon. Treatment depends on the degree of deviation. If minimal, a conservative and expectant attitude, based on prevention and treatment of possible complications, is recommended because of the possibility of spontaneous regression of the nail deviation. Surgical therapy may be considered in patients with severe or complicated forms.