Coloboma is one of the congenital ocular malformations presenting important diagnostic and therapeutic problems, especially in infants. Coloboma can be associated with microphthalmia frequently and macrophtalmia rarely. However, the association of coloboma, macrophthalmia, and orbital cyst was not reported previously in the literature. A case of coloboma associated with macrophtalmia and cyst was presented, and MR findings of this anomaly was defined with emphasis to the importance of radiologic examinations in the diagnosis of some congenital ocular malformations. (c) 2005 Elsevier Inc. All rights reserved.