Chorea-acanthocytosis (ChAc) and McLeod syndrome (MLS) are the progressive neurodegenerative diseases with the rare incidence [1]. Although the dysfunction in XK-scramblase results in MLS, the origin of ChAc is the VPS13 A (vacuolar protein sorting-associated protein 13 A) mutations, leading to neurodegeneration and dysfunction of lipid metabolism [2]. Based on the peripheral blood smear (PBS), the acanthocytes cells are identified as a few spicules with different sizes which project from the surface of RBC membrane with the irregular intervals [[3], [4], [5]]. These alterations occur in the membrane lipids and proteins. Consequently, the acanthocytosis can be detected with the severe liver dysfunction, neuroacanthocytosis, abetalipoproteinemia, malnutrition, hypothyroidism, post-splenectomy [6]. ChAc is frequently distinguished by the presence of acanthocytes and neurological symptoms [7,8]. It is estimated that 500–1000 male or female involve with ChAc in all over the world. The involuntary movement disorder with orofacial dyskinesia and progressive cognitive decline are observed in the ChAc patients which may be affected by age [9,10]. In few cases, seizures may overtake from the movement disorders, even a decade. There is an unmet need to early prevent the syndrome complications in such cases. The reported case is an unusual familial ChAc in a 44-year-old male who presented an episode of generalized tonic-clonic seizure with a 13-year history of gradually progressive abnormal movements, and generalized weakness. Although the patients involved with ChAc are unlikely to be treated effectively, the aim of article is to sensitize readers in treatment of cases presenting generalized tonic-clonic seizure.