RCCs are an infrequent cause of symptoms in pediatric patients. The transsphenoidal approach offers an effective means of achieving complete cyst drainage for lesions requiring surgery. Fenestration and aspiration of the cyst are usually sufficient to achieve total resolution of symptoms and signs caused by RCCs. Clinical symptoms such as headaches improved in the majority of patients, whereas hormonal disturbances typically persisted. Patient selection remains of paramount importance when considering surgery for pediatric patients with RCCs.