Aplasia cutis congenita (ACC) is a rare disorder that is estimated to affect approximately three newborns in every 10,000 live births. It is usually detected at birth and most commonly affects the scalp as a solitary lesion. The type of lesion may be classified into one of nine groups. Membranous types and those with a “hair collar” are at highest risk to be associated with underlying neurodevelopmental abnormalities. In rare cases, associated abnormalities and syndromes may also be seen. Appropriate evaluation can be undertaken depending on clinical signs and symptoms. Treatment is often conservative, but more aggressive treatment may be indicated for some larger scalp lesions.