Amyotrophic lateral sclerosis (ALS) is characterized by progressive onset motor deficits with heterogenous presentations ranging from dysarthria to foot drop. Approximately 20% of the patients present with focal bulbar symptoms, in which some may remain restricted to bulbar region (isolated bulbar palsy), and the remaining eventually spreads to involve other body regions (classical ALS). Without accompanying upper and lower motor neurons signs elsewhere, differential diagnoses for isolated bulbar symptoms are extensive, include ALS variants as well as potentially treatable mimics. Therefore, it is important to take heed on every possible aetiology that may disrupt the hypoglossal nucleus, nerve, or lingual muscle itself. Herein, we illustrated a rare presentation of Group A basilar invagination, which mimicked bulbar-onset ALS.