Chondrodermatits nodularis chronica helicis (CNCH), first described by Max Winkler in 1915, presents as a sore nodule on the helix or antihelix of the external ear. In this paper, we review the etiopathogenesis and management options of CNCH. This condition has a multifactorial etiology; however, sustained pressure from sleeping on one side is the favored theory. Currently, there are many surgical and non-surgical methods of treating CNCH. Most practitioners recommend conservative measures first in their patients, such as pressure-relieving prostheses, prior to surgical treatment. Surgery is the gold standard of therapy with cartilage and wedge excisions yielding recurrence rates of about 10%. Carbon dioxide laser and photodynamic therapy are newer treatment modalities for CNCH, yet they have recurrence rates similar to conservative therapy. In conclusion, due to the high rates of CNCH recurrence, wedge resection is the suggested treatment for CNCH after conservative measures fail.