This case report details a perineal hernia in a 3‐year‐old Angus heifer in late gestation. The heifer had a gradually increasing swelling of the perineal region since 2 weeks. Upon palpation, the swelling was painless, hard and non‐reducible. Ultrasonographic examination revealed a hernial sac enclosing the non‐pregnant horn of the uterus, filled with homogenous hypoechoic fluid. Based on the anamnesis and clinical examination, a tentative diagnosis of perineal herniation was made. Considering the deteriorating clinical condition of the cow and the possible risk of dystocia, the viable calf was delivered by Caesarean section. The cow recovered with a reducible hernial sac, which was decided not to correct surgically due to its large size. After 7 days, the cow showed no further complications and was discharged. In conclusion, although rare, a perineal hernia involving the uterus should be considered in the differential diagnosis of perineal swellings in pregnant cows.