Practical relevance: Although a relatively uncommon condition, the investigation, diagnosis and initial medical management of feline congenital portosystemic shunts is often undertaken within general practice. Early recognition and appropriate treatment are important to ensure a good outcome. Clinical challenges: Clinical signs associated with CPSSs in cats are extremely variable and can be intermittent. Signs can affect a variety of organ systems including the nervous system, and gastrointestinal and urinary tracts. Thus, the differential diagnosis list may be very long and a CPSS may not be suspected initially. More specific diagnostic tests and diagnostic imaging are indicated but may not be 100% accurate and may not be readily available to the general practitioner. Audience: This review highlights challenging aspects of the investigation and management of CPSSs for the practising veterinarian, but is also relevant to postgraduate students and provides a practical summary for specialists. Patient group: In practice, domestic shorthairs make up the majority of cats with CPSSs. However, Siamese, Persian and Himalayan cats may be more commonly affected than other purebreeds. While cats with CPSSs are typically under 6 months old, the condition is seen in mature animals, which may not have exhibited clinical signs for months or years. Evidence base: Despite several retrospective studies of cats with CPSSs, the evidence base for management of the condition remains limited.