A case of neonatal manifestation of giant hypothalamic hamartoma is re
ported. It is suggested that hypothalamic hamartoma should be included
in the list of neonatal intracerebral tumors. Magnetic resonance imag
ing appearance similar to that of normal gray matter on T1-weighted im
ages and slightly hyperintense on T2-weighted images, without enhancem
ent after gadolinium injection, is suggestive of the diagnosis. Hypoth
alamic hamartomas are congenital malformations, consisting of disorgan
ized mature neuronal elements in proportions similar to that of normal
tissue [1]. They are clinically evidenced in infants ranging from 1 t
o 7 years of age [1-5]. This report describes a histologically proved
giant hypothalamic hamartoma diagnosed in the neonatal period. Magneti
c resonance imaging (MRI) is helpful to distinguish this congenital no
n-evolutive malformation from more aggressive neonatal tumors.