We report the first family from the Arabian Peninsula harboring a novel frameshift mutation in the SACS gene. CASE SUMMARY: A 33 year old man presented to our neurology clinic with balance problems and weakness of distal upper and lower limbs. ...Close examination of other family members identified cerebellar ataxia, lower limb pyramidal signs, peripheral neuropathy, and magnetic resonance imaging findings characterized by pontine linear hypointensities. ...