On 1 October 2001, a 4-mo-old male white-tailed deer (Odocoileus virginianus) fawn was collected in Day County, South Dakota (USA), by South Dakota Department of Game, Fish and Parks personnel. The fawn had sparse hair development on the ventral thorax, the lateral caudal and caudal aspects of the rear legs, the muzzle, around the eyes, and inside the ears. Remaining skin surfaces were devoid of hair. Histologic examination revealed normal hair follicle density although follicles were empty or contained keratin debris and fragments of hair shaft. The epidermis of the fawn was mildly thickened and melanin pigment was prominent within deep layers of the epidermis. Based on histologic examination, the deer was diagnosed with congenital hypotrichosis. Although this condition has been reported in domestic species and humans, this specimen represents the first documented case of congenital hypotrichosis in a cervid.