Purpose: To describe the management and outcome of a patient with optic disc coloboma-associated cavitary disc maculopathy treated with optic nerve sheath fenestration and adjunctive juxtapapillary laser photocoagulation. Methods: A single case was evaluated. Results: A 20-year-old woman with a history of deep amblyopia in the right eye presented with total serous retinal detachment associated with optic disc coloboma. The patient underwent optic nerve sheath fenestration as the primary treatment. One month after surgery, adjunctive juxtapapillary laser photocoagulation was performed to create a barricade and reduce the risk of recurrent subretinal fluid accumulation. Visual acuity remained stable at 20/400 in the affected eye. Following optic nerve sheath fenestration and laser treatment, the retinal detachment resolved completely. Conclusions: Optic nerve sheath fenestration combined with juxtapapillary laser photocoagulation may be an effective therapeutic approach for managing select cases of optic disc coloboma with associated cavitary disc maculopathy.