Cerebrotendinous xanthomatosis (CTX) is a rare auto somal recessive lipid storage disorder caused by mutations in the CYP27A1 gene, resulting in deficiency of sterol 27-hydroxylase, a key enzyme in bile acid synthesis. Impaired bile acid metabolism leads to accumulation of cholesterol and cholesterol in multiple tissues, particularly the brain, tendons, and eyes. We report a case of a 32-year-old male who presented with multiple symmetrical tendon swellings involving the Achilles tendons and elbows, along with progressive lower limb stiffness and a history of early-onset cataracts. Magnetic resonance imaging (MRI) of the brain demonstrated bilateral T2/FLAIR hyper intensities involving the cerebellar dentate nuclei and per ventricular white matter. Fine needle aspiration cytology (FNAC) from the tendon swellings revealed numerous foamy macrophages, multinucleated giant cells, and cholesterol clefts in a lipoidal background, consistent with xanthomatous pathology. Biochemical evaluation was suggestive of CTX. This case highlights the diagnostic value of FNAC as a simple and minimally invasive adjunct, particularly when correlated with clinical and radiological findings, and underscores its usefulness in resource-limited settings for early recognition of CTX.