BackgroundGall bladder mucoceles (GBM) are a leading cause of biliary disease in dogs with several breeds, including the Shetland Sheepdog, American Cocker Spaniel, Chihuahua, Pomeranian, and Miniature Schnauzer apparently predisposed.ObjectiveTo determine risk factors, clinical features, and response to treatment of GBM in Border terriers (BT).AnimalsMedical records of 99 dogs (including 51 BT) with an ultrasonographic (±histopathologic) diagnosis of GBM from three referral centers in the United Kingdom were collected. A control group of 87 similar‐aged BT with no ultrasonographic evidence of gall bladder disease was selected for comparison.MethodRetrospective case‐control study. Odds ratios were calculated to establish breed predisposition. Signalment, presence of endocrine disease, clinicopathologic results, and outcome were compared between the BT, other breeds, and control BTs.ResultsThe odds of identifying a GBM in a BT in this hospital population was 85 times that of all other breeds (95% confidence interval 56.9‐126.8). BT had similar clinical signs and clinicopathologic changes to other breeds with GBM. There was no evidence that endocrinopathies were associated with GBM in BT.Clinical SignificanceA robust breed predisposition to GBM is established for the BT.