Introduction: Although craniopharyngiomas are congenital tumors, they are rarely diagnosed in the prenatal or early neonatal period. Sometimes they are very large, debilitating and life-threatening, as in the case discussed here.
Observation: A 21-month-old girl was referred for craniopharyngioma that had been diagnosed at 5 months because of neurologic complications. The pregnancy and birth were considered to be unremarkable. The birth weight was 4 kg, length 53 cm, head circumference 38 cm (n=35±1). Brain CT revealed active hydrocephalus caused by a very large solid, cystic and calcified supra sellar mass. MRI confirmed the process measuring 5 cm in height, multidirectional, diving into pituitary sella, displacing the brainstem. After several resections and reservoir drainage, the patient was blind, hemiparetic and she convulsed frequently. The patient died when she was 2.
Conclusion: The large head circumference at birth, the size of the tumor, blindness, and hemiparesis observed before the age of 5 months suggested an antenatal process unnoticed because of inadequate gestational monitoring.