An infant with bilateral congenital microphthalmos with cyst developed neurologic and cardiac abnormalities that resulted in her death at the age of 7 months. When she was first examined, the cystic masses in both eyes transilluminated and were easily deformable. No ocular structures could be identified. Bilateral microphthalmos with cyst appears to be associated with major systemic abnormalities whereas unilateral microphthalmos with cyst is associated with much less serious systemic abnormalities.