A 9-year-old boy with cyanotic congenital heart disease demonstrated congenital horizontal gaze paralysis and dysplasia of the left external and middle ear. Another patient associating congenital horizontal gaze paralysis and left hemifacial atrophy including ear dysplasia had previously been reported. The coincidence of such rare dysgenetic phenomena as congenital horizontal gaze paralysis and ear dysplasia in 2 patients seems to be significant. Since patients with congenital horizontal gaze paralysis often replace lateral gaze by convergence spasms, gaze paralysis sometimes remains undiagnosed. Therefore, when ocular motor disturbance is associated with ear dysplasia, the possibility of congenital horizontal gaze paralysis should be considered.