Congenital ocular malformation may lead to failure of the development of ocular regions and visual function in the pediatric population. Orbital bones are established within the first 2 months of embryogenesis. Any abnormalities may lead to failure in development of the ocular region. In this case report, we present a newborn with congenital canthus malposition, a distorted tarsus, and mild enophthalmos caused by an embryogenesis fibroma, which although rare, can result in amblyopia. Surgical resection of the fibroma followed by orbital reconstruction of the canthus, correction of malposition and tarsus extension were performed. Twelve months and 5 years follow-up showed no recurrence and the visual acuity was 20/20 in the left eye and 16/20 in the right. The deformity of the left orbit was corrected without complications and was symmetrical with the right side. This is the first known case of children with embryonic fibroma. It is likely that early reconstruction surgery may avoid visual occlusion and ocular malformation.