Congenital horizontal gaze paralysis is a rare disorder which may be a
ssociated with severe scoliosis of early onset. We present the clinica
l findings of two sisters with this syndrome. The additional finding o
f a unique pericentric inversion of chromosome 12 is considered to be
incidental. Every child with congenital horizontal gaze paralysis shou
ld be evaluated for a possibly associated scoliosis. If present, a dia
gnosis of this presumably autosomal recessive syndrome can be made wit
h appropriate treatment and genetic counseling.