Riga-Fede disease: association with microcephaly

A case of microcephaly associated with traumatic ulceration to the ventral surface of the tongue (Riga-Fede disease) in a 12-month-old female is presented. To the best of our knowledge, such association has not been described previously. A conservative treatment regime was used, involving medical management, elimination of the sharp edges of the teeth and use of topical triamcinolone, and the ulceration healed over a period of 4 weeks.