Foetal alcohol spectrum disorders are a group of conditions related with the prenatal exposure to alcohol. Affected subjects may manifest a wide variety of birth defects (growth retardation, craniofacial dysmorphisms, malformations) and neurodevelopmental impairments (cognitive, speech, memory and learning disorders). Such anomalies have, as secondary effects, an increased risk of adverse social (school dropout, increased frequency of crimes, reduced ability to work and independent living) and clinical (psychiatric diseases, substances/drugs addiction) outcomes, whose impact could be limited by early diagnosis and treatment. The global prevalence is currently estimated at 7.7 per 1,000 children and young adults (range 4.9-11.7 per 1,000), but the rate is 10 to 40 times higher in at risk population groups (prisons, foster homes). The lack of biological markers or objective tests for FASD diagnosis explains the use of clinical based diagnostic guidelines. Despite a widespread and strong awareness of FASD as a distinct clinical entity, no globally standardized diagnostic criteria are available until now. Accurate and timely diagnosis is necessary to provide the most suitable treatment aimed to improve patients’ and families’ health outcomes, as well as to adequately address scientific research and preventive strategies. This review has been conducted analysing the most recent studies and is proposed as guide and compendium of the main aspects that the paediatrician has to know for a correct diagnosis and an adequate management of patients.