We report a case of a 33 years old female with a history of paroxystic hemidystonia treated by acetazolamide, a carbonic anhydrase inhibitor (CAI), and who developed two years after the initiation of this treatment bilateral radio-opaque stones. Laboratory tests revealed a hyperchloremic acidosis, an elevated urinary pH, a hypercalciuria, a severe hypocitraturia and numerous granulations of amorphous carbonated calcium phosphates and brushite crystals on urinary microscopic examination, the whole suggests a diagnosis of acetazolamide-induced nephrolithiasis. We discuss in this article the lithogenetic process and the usual composition of the stones induced by CAI, and specific risk factors for developing drug-induced lithiasis which should be taken into consideration when prescribing long-term drug regimens.