A 30 year old male presented with insidious onset of painless swellings in tendons over 20 years, with learning difficulty, tremors, progressive instability of gait and frequent falls. There was no similar illness in family. On examination, there was firm, non tender, nodular swelling Achilles, patellar and tricep tendon, clubbing of fingers. The Mini Mental Status Examination score was 16/30. Plantar response was extensor. There was cerebellar signs with nystagmus, truncal titubation, ataxic gait and intentional tremors. Cranial MRI showed bilateral hyperintensities in dentate nucleus, globus pallidus, periventricular white matter on FLAIR/T2 sequences, and diffuse cerebral and cerebellar atrophy. The patient was diagnosed as having cerebrotendinous xanthomatosis based on the clinical and radiological features. As cerebrotendinous xanthomatosis benefit from therapy and progress of the disease is preventable, the case illustrates the need for early diagnosis.