INTRODUCTION: Peyronie's disease (PD) is a wound-healing disorder of the tunica albuginea often associated with penile deformity. Less commonly, patients with PD might display atypical presentations such as ventral curvature, hourglass deformity, significantly shortened penis, and/or multiplanar curvature. AIM: To review the available literature on the prevalence of and management options for atypical PD. METHODS: A literature review was performed through PubMed from 1982 through 2016 regarding atypical PD. Keywords used for the search were Peyronie's disease, atypical Peyronie's disease, ventral Peyronie's disease, ventral plaque, hourglass deformity, penile indentation, penile notching, short penis, shortened penis, shrunken penis, biplanar curvature, and multiplanar curvature. MAIN OUTCOME MEASURES: To assess the various surgical and non-surgical techniques used for the management of atypical PD. RESULTS: Collagenase Clostridium histolyticum is contraindicated for patients with ventral plaques and/or hourglass deformities. Patients with maintained erectile function and ventral PD plaques are best treated with intralesional injections of interferon alpha-2b or tunica plication. Patients with maintained erectile function with PD associated with hourglass deformity and/or multiplanar curvature are best treated with plaque incision or partial excision and grafting. Patients with a shortened penis could attempt conservative measures such as penile traction therapy and medical management. When erectile function is compromised, insertion of an inflatable penile prosthesis with or without ancillary straightening procedures should be recommended. Lengthening procedures can be attempted in very special circumstances by expert surgeons. CONCLUSION: There is a paucity of data regarding atypical PD. Ventral plaques can be treated with intralesional injections or surgery, hourglass deformity and multiplanar curvatures are best managed surgically, and a shortened penis should be treated with non-invasive approaches. When concomitant erectile dysfunction is present, insertion of an inflatable penile prosthesis is recommended. Caution should be advised before undergoing ancillary penile lengthening maneuvers owing to the potential for serious complications. Yafi FA, Hatzichristodoulou G, DeLay KJ, Hellstrom WJG. Review of Management Options for Patients With Atypical Peyronie's Disease. Sex Med Rev 2017;5:211-221.