AbstractLate recurrences of first‐stage inflammatory vesiculobullous lesions of incontinentia pigmenti (IP) are uncommon but have been reported to occur in the setting of infections, fevers, and vaccinations. This phenomenon has not been described in the setting of atypical hand, foot, and mouth disease (HFMD). In addition, atypical HFMD often has a predilection for areas of previous inflammation or trauma, such as areas of atopic dermatitis (eczema coxsackium). We present a case of incontinentia pigmenti coxsackium in which a coxsackievirus infection triggered reactivation of IP and cutaneous viral lesions were concentrated in areas of IP.