Spontaneous and sequential corneal perforation is rare in pellucid marginal degeneration (PMD) in the seventh decade of life. Hence, the importance of repeated follow-up, clinical examination along with serial corneal topography even in the seventh and eighth decades of life is of utmost importance in PMD. We report a case of spontaneous corneal perforation in a 64-year-old male patient with PMD having normal corneal thickness in the right eye which was managed with primary suturing and compression sutures. The other eye was absolutely normal with normal topographic findings. Over a period of four years of follow-up, the patient developed progressive diminution of vision in both eyes and was diagnosed with cataract. Temporal phacoemulsification with toric IOL implantation was done in both eyes, following which the left eye developed spontaneous perforation after a period of two months which was also managed with primary suturing and compression sutures.