Purpose: To report the optical coherence tomography (OCT) findings in a patient with unilateral macular coloboma. Methods: A 12-year-old male was presented with macular coloboma in the left eye. The optical coherence tomography was performed with fluorescein angiography (FA). Results: The OCT revealed the crater-like depression in the macula, demonstrating atrophic neurosensory retina, and an absence of retinal pigment epithelium and choroid in the lesion. FA showed hypofluorescence corresponding to the size of the lesion in both early and late frames without leakage of dye at any stage. Conclusions: The OCT can be beneficial to confirm the diagnosis of macular coloboma.