Background: The two most important goals of management of keratoconus and other corneal ectatic diseases are halting disease progression and visual rehabilitation. Several treatment strategies to skip corneal transplantation have been developed but controversies of the best treatment option for a given patient still exist. The combination of CXL and PIOL implantation has been proposed for visual rehabilitation in patients with progressive keratoconus. Aim: To review the published clinical evidence on the combination of corneal cross-linking (CXL) and phakic intraocular lenses (PIOLs) in patients with keratoconus. Results: No randomized controlled trials and only four retrospective case series were identified. The progression of keratoconus was stopped in all eyes and satisfactory visual rehabilitation was achieved both in terms of uncorrected and corrected distance visual acuity (CDVA) and predictability of refractive correction. Conclusion: Corneal cross-linking combined with PIOL implantation is a valid therapeutic approach for progressive keratoconus with moderate-to-high refractive errors, regular or mildly irregular astigmatism, and good CDVA, especially in the face of significant anisometropia. Clinical significance: The combination of CXL and PIOL implantation is a valid therapeutic approach for visual rehabilitation of progressive keratoconus. Although longer-term follow-up clinical data from prospective randomized clinical trials (RCTs) are needed, clinical outcomes are excellent and equivalent to nonkeratoconic eyes up to 3 years after surgery.