Pentosan polysulfate maculopathy

•Long-term use of pentosan polysulfate (trade name Elmiron) can cause maculopathy.•Incidence is dose dependent with a 1,818 g median cumulative dosage in reported cases.•May result in prominent macular dysfunction, especially in cases with RPE atrophy.•Multimodal imaging including fundus autofluorescence is key in diagnosis.•Routine annual screening is suggested for all patients using PPS.