Introduction: Congenital aniridia is a rare disease associated with absent irides and various other ocular and systemic conditions. We report an extremely rare incidence of congenital aniridia associated with congenital aphakia and glaucoma in a young man. Case description: A 28-year-old man presented with symptoms of ocular discomfort and low vision in his left eye. Visual acuity was finger counting at a half-meter, and examination showed the presence of aniridia, aphakia, nystagmus, and aniridic keratopathy, along with partial glaucomatous optic atrophy, poor foveal reflex, and a few white vitreous opacities in the fundus. The management was done conservatively with aphakic glasses, lubricants, and antiglaucoma drops. Conclusion: The management of congenital aphakia associated with other ocular anomalies should be aimed at the preservation of residual vision and prevention of sight-threatening complications.