In our patient, the overlapping features with those of TET3-BEFAHRS were development delay, in particular, speech, gross motor and fine motor delay, mild intellectual disability, tall stature (height above the 97th centile), macrocephaly (OFC 61 cm), normal weight (3–25th centile), movement disorder (ataxia), scoliosis, the pattern of ESES, which was described in two other patients as a unique pattern associated with a crisis, and seizures refractory to standard therapy, as in our patient [1,2] and in one patient with complex partial epilepsy [3]. The gene discussed is TET3; the disease is cerebellar ataxia.