Based on own clinical experiences and the literature, this review article elaborates on aspects of aetiology, pathogenesis, clinical signs, diagnosis and therapy of haemophilia A and B in dogs. When compared to humans, dogs reveal more severe haemorrhagic symptoms at a defined residual factor activity (e.g. subcutaneous, intramuscular, and intraarticular haemorrhages after inappropriate trauma resulting in lameness and paralysis, excessive haemorrhage during second dentition, venal puncture, and surgery). Fortunately, genetic tests are now commercially available in Germany for selected breeds (haemophilia B in Rhodesian Ridgebacks; haemophilia A in Havaneses), which complement the conventional individual factor activity measurements and facilitate the detection of female carrier dogs. Treatment of bleeding crises is still mainly based on substitution therapy with fresh or fresh frozen plasma in addition to local haemostatic measures. In contrast, expectations regarding the timely clinical availability of gene therapy (particularly in humans) have not yet been fulfilled.