Perilymphatic fistulas of the inner ear constitute abnormal leaks of perilymphatic fluid into the middle ear or mastoid air cell system and represent a rare cause of otorrhea. We report the case of a 5-month-old child presenting with sudden otorrhea. High resolution CT cisternography showed a malformation of the middle ear and a passage of contrast-enhanced CSF into the tympanic cavity through the left oval window. Surgery confirmed a tear of the tympanic membrane as well as a stapes malformation with aplasia of the crura and an associated perilymphatic fistula through a defect in the stapes footplate. The fistula was closed with adipose tissue from the ear lobe.