The preauricular sinus is a not uncommon finding in the paediatric population. Recent reports have added to our knowledge of this benign malformation. We review the current literature with respect to the aetiology of the condition, its clinical features, and associations with other congenital malformations. In those patients in whom a preauricular sinus is identified, we recommend associated congenital anomalies be sought. In selected cases, a renal ultrasound scan may be appropriate. Where no associated abnormalities are identified, and where the preauricular sinus is asymptomatic, there is consensus opinion that no further action is indicated. In the acute phase of infection, treatment comprises administration of appropriate antibiotics, and incision and drainage of an abscess if present. In the symptomatic preauricular sinus exhibiting recurrent or persistent infection, opinion regarding optimal management varies. Latest evidence suggests definitive surgical treatment offering the most favourable outcome is by wide local excision of the sinus, as opposed to the previously preferred technique of simple sinectomy. Magnification employed during surgery, and opening, and following from the inside as well as outside, branching tracts of the sinus may further minimise the risk of recurrence.