Pimozide is an antipsychotic drug of the diphenylbutylpiperidine class. In the US, it is FDA-approved only as a backup treatment for Gilles de la Tourette syndrome, although it has been used in other countries for many years as a treatment for schizophrenia. In the past 20 years, pimozide has been found to be especially efficacious in the treatment of monosymptomatic hypochondriacal psychoses and is used by psychiatrists and dermatologists for this off-label purpose. In particular, pimozide is considered the treatment of choice for delusions of parasitosis. In addition, pimozide has been found to be efficacious in the treatment of body dysmorphic disorder, metastatic melanoma, trichotillomania, and trigeminal and postherpetic neuralgia. This review aims to familiarize physicians, especially dermatologists, on the uses of pimozide in dermatologic practice. A review of the literature was performed and the relevant information synthesized to give a complete overview of the drug and its therapeutic uses in dermatology.