Cushing disease is a state of endogenous glucocorticoid over-production, resulting from pituitary corticotroph hypersecretion. The most sensitive indicator of excess glucocorticoid secretion in the pediatric age group is growth failure, which precedes other classic manifestations of Cushing disease. Transsphenoidal surgery is advocated by many as the treatment of choice in children and adolescents. However, disparate “cure” rates are reported, ranging from 50 to 98%. The discrepancies may be explained by differences in the skill of the neurosurgeon, and by the length and method of follow-up. At UCSF, forty-two consecutive children or adolescents (age ≤ 18 yrs) underwent transsphenoidal exploration for the primary treatment of Cushing disease from 1974 to 1993. Six patients had persistent disease(evidence of Cushing disease within 6 months of surgery). Of these, four required bilateral adrenalectomy, one had repeat transsphenoidal surgery, and one continues to have evidence of Cushing disease. We have comprehensively evaluated twenty-six of the remaining thirty-six patients, including testing of the ACTH/adrenocortical axis. Follow-up range is 1.5 - 13.6 years, with a mean 7.2 yrs. At six months to five years of follow-up, 22 patients remained in remission. Of the sixteen who were followed for five to nine years, two developed a recurrence. Of the 11 followed for greater than 9 years, none have developed recurrences. Recurrence was documented in a total of six patients, with a range of 9 mos to 6.2 yrs post-op, with a mean of 4.2 years. Of the patients in whom we have final height data (n=19), height at the time of diagnosis was 1.7 standard deviations below the mean. Final height was 1.14 SD below the mean. Midparental target height was + 0.4 SD. CONCLUSION: In children and adolescents treated by transsphenoidal adenomectomy for Cushing disease and followed for greater than six months, with a mean of 7.2 years, the overall remission rate is 77%. Transsphenoidal surgery remains the optimal therapy for the initial treatment of Cushing disease in children and adolescents. The final height measurements show that the growth retardation caused by cortisol excess in Cushing disease, despite catch-up growth following surgical cure, can be associated with a compromised final adult height.