Penile strangulation by hair coil is an uncommon, under-recognized condition with a spectrum of potential complications including urethral transection and partial or complete penile amputation. Early recognition and treatment can greatly reduce the incidence of these devastating complications. We report a 5-year-old, circumcised boy with penile edema and erosions secondary to a hair wrapped around the coronal sulcus. Prompt diagnosis and removal of the hair led to rapid resolution without surgical repair. We also analyze the possible underlying causes which can lead to strangulation of the penis with a hair.