We present the case of a 9-year-old female with developmental delay, hypotonia, and Lennox-Gastaut syndrome who presented to the movement disorder clinic for a wide range of motor and vocal tics. She had an uncomplicated birth, but seizure onset at 6 months, and severe delay of speech and motor milestones. She began having blinking, eye deviation, and hand shaking around age 4yo. Over time, tics included blinking, arrhythmic facial contractions, humming, compulsively touching the elbow, and complex sequences including chin protrusion- head nodding- body shivering, and stirring movements of the arms followed by throwing back the flexed forearms in rapid succession. She also experienced significant irregular, jerky tremors of the limbs that interfered with activities. She had a wide-based, spastic and ataxic gait with marked anteropulsion. Behaviorally, she had compulsive tendencies of lining up all of her stuffed animals in a particular way and demanding that game sequences repeat the same pattern as the last time played. Work-up included an MRI with cerebellar, especially vermal atrophy. Chromosomal microarray, fragile X testing, and epilepsy gene panel were negative. Whole exome sequencing found her to be heterozygote for KCNA2 mutation (autosomal dominant), R297Q variant with specific mutation c.890G>A.