The purpose of this paper is to present a case of an 18-month-old girl initially presenting with strabismus. Fixation of the affected eye was intermittent with a relative afferent pupillary defect. A fundus photography of the affected left eye showed a combination of features of both morning glory disc anomaly (MGDA) and peripapillary staphyloma. MGDA is a congenital malformation of the optic nerve, characterised by a funnel-shaped excavation of the posterior globe that incorporates the optic disc. Peripapillary staphyloma is a rare congenital optic disc anomaly in which a deep fundus excavation surrounds a relatively normal appearing optic nerve head. Peripapillary staphyloma is rarely associated with other congenital defects or systemic diseases. No further systemic associations with the disease have been discovered so far in this individual patient. Cycloplegic refraction measurements revealed a large amount of anisometropia. Treatment included refractive correction and part-time occlusion. The authors support the theory that MGDA and peripapillary staphyloma may represent two different morphologies in the spectrum of the same disease. Regular follow-up is recommended to allow early treatment of complications and timely diagnosis of any associated general health problems.