Granular cell tumor (GCT) usually occurs as a solitary, small, nodular tumor and rarely grows to a sufficient size to present symptoms. The tumor is more prevalent in female adults. The authors report a case of an 8-year-old boy with GCT of the neurohypophysis presenting with central precocious puberty. Few cases of symptomatic GCT have been reported in the literature, none of them in a patient in the first decade of life. Clinical picture, histopathological features, and histogenesis of GCT of neurohypophysis are discussed.