Purpose: Introduce VACTERL deformities to the ophthalmologic literature, report the case of VACTERL deformities associated with congenital limbal dermoid and to review the ocular and systemic findings that may occur in this group of disorders. Methods: Evaluation of a child with VACTERL syndrome associated with congenital limbal dermoid and observation of the ophthalmologic and systemic manifestations. Results: We report a case of a six years old male with mass on limbus with a hair follicle in it in the right eye since birth. He also had hemi vertebrae in the lower cervical and upper thoracic region, seven carpel bones, congenital anorectal anomaly for which he got operated at the age of two years, congenital inguinal hernia and VSD. Surgical correction of inguinal hernia was done. We posted him for excision of limbal dermoid. Conclusion: Ophthalmologists who examine children with limbal dermoid should consider VACTERL syndrome in the differential diagnosis and should obtain appropriate radiological investigations and imaging studies when the diagnosis is suspected. Congenital limbal dermoid should be added to the list of VACTERL syndrome associated to ocular anomalies.