Isolated unilateral hypoglossal nerve palsy (HNP) is uncommon with only a few case reports and two case series in literature. Our study is the largest case series including 12 patients. Till date isolated HNP was considered an ominous clinical sign indicat- ing an underlying malignancy, however ,in the present case series treatable causes such as tubercular central nervous system infection(33%)and idiopathic (25%) cases overweighed the neoplastic (16%) etiology. Sarcoidosis (8%), atlanto occipital disloca- tion (8%) and aberrant ectatic vessel in the hypoglossal canal (8%) were contributory in the remaining cases.60% of the patients had a signifcant clinical recovery during follow up. The present study emphasizes that isolated HNP does not always herald a grave prognosis and an extensive diagnostic workup in these cases may yield common and potentially treatable etiologies.