This report describes a rare case of spontaneous Grade-4 retinal pigment epithelium (RPE) rip of serous pigment epithelial detachment (PED) in central serous chorioretinopathy (CSC) and RPE aperture in the fellow eye, with favorable long-term outcomes. A 38-year-old man presented with defective vision (20/30) in the left eye (LE) due to bullous CSC associated with a large extramacular RPE rip located temporally and inferior exudative retinal detachment. Optical coherence tomography (OCT) confirmed a subfoveal serous PED with RPE aperture, subretinal fluid (SRF) and fibrinous exudation, and a large extramacular RPE rip temporally. The right eye (RE) had an asymptomatic large serous PED. The LE was treated with low-fluence photodynamic therapy, which resulted in the closure of RPE aperture and complete resolution of PED and SRF. Six-month later, the patient presented with sudden defective vision (20/120) in the RE secondary to a large fovea-involving (Grade-4) RPE rip with SRF as confirmed on OCT. Fluorescein angiography showed two extrafoveal active point leaks, which were treated with focal photocoagulation. He was also started on oral eplerenone. On subsequent serial follow-ups over 1 year, OCT showed SRF resolution and patchy reorganization of the subfoveal RPE-photoreceptor complex with good visual outcome (20/30).