Oral-facial-digital syndrome was first described by Papillon-League and Psaume in 1954, and since then, up to 9 distinct types of OFDS have been described. Ocular findings that have been described include hypertelorism in types I, II, III, IV, and VI; strabismus in I, II, and IV; and see-saw winking in type IV.1 Recently, a new type (originally described as type VIII, but reclassified as type IX) has been described as a variant of type II associated with retinal abnormalities.2 To date, this new type IX has been the only type associated with intraocular findings. However, it is unclear whether complete ophthalmologic examinations were performed on the early reported cases of OFDS; intraocular anomalies therefore cannot be excluded in other types of OFDS. Our patient has findings most consistent with OFDS type IX. To date, only 6 prior cases of OFDS type IX have been reported, including 5 cases of chorioretinal colobomas and 1 case of optic nerve coloboma. To our knowledge, retinal masses, such as the ones in our patient, have never been reported in OFDS.