Two Friesian cows, half-sibs by a common dam, produced four bull calves with severe congenital hypotrichosis and incisor anodontia and three normal heifers by six unrelated Holstein, Friesian, Devon and Hereford bulls. The two dams, and their dam, had coats of a short, stubbly nature and the pigmented areas appeared rusty grey rather than black. Pathological examination of skin samples taken from multiple standardised sites from two of the affected calves showed a reduction in the number of large first-formed hair follicles. Smaller calibre hair follicles were present but, unlike those of normal neonatal calves, all were in the telogen (inactive) phase. Although the herd was infected with bovine virus diarrhoea virus there was no convincing evidence that the virus was implicated in the pathogenesis of these cases of congenital hypotrichosis with incisor anodontia. The family breeding information on the few severely affected and normal progeny and mildly affected and normal parents may be explained by the genetic hypothesis of an X-linked incompletely dominant gene.