Cystic fibrosis (CF) is a spectrum of disease from single organ manifestation and mild symptoms to multiple organ involvement with severely impaired function. Patients with pancreatic sufficiency often present later in life as their nutritional status is preserved. In most cases the diagnosis is straightforward and is confirmed by a sweat chloride concentration above 60 mmoL/L and/or first-line genotyping. In an important minority, and often patients presenting in adulthood, more extensive testing is required, including full gene sequencing and nasal potential difference measurements. Regardless of the age at diagnosis, the management principles are the same, as there is a significant risk of progression. Care should be delivered by a multidisciplinary team from an accredited CF center. CF-specific therapies should be initiated and other important aspects of care, such as genetic counselling, should be offered.