<h4>Purpose</h4>We report the case of a neurologically asymptomatic young boy presenting with an unusual phenotype of <i>CYP2U1</i> related macular dystrophy associating bilateral macular telangiectasia (MacTel) and fibrotic choroidal neovascularization (CNV), assessed with complete multimodal imaging including optical coherence tomography angiography (OCT-A).<h4>Case presentation</h4>A twelve-year-old boy from a non-consanguineous family complained of bilateral progressive visual loss and photophobia. The gene discussed is CYP2U1; the disease is macular telangiectasia type 2.