A healthy 18-month-old girl presented with a history of intermittent hemifacial flushing when eating. Her symptom seemed to be exclusively triggered by chewing. Examination revealed cutaneous features of neurofibromatosis type 1 (NF1) and mild facial asymmetry. Imaging confirmed cerebral vacuolisation changes seen in NF1 and a left facial plexiform neurofibroma involving the parotid gland. This is the first reported case of Frey syndrome complicating NF1.