Acrodermatitis continua of Hallopeau (ACH) is a rare variant of pustular psoriasis that presents as a sterile pustular eruption of the periungual and subungual regions. It affects the skin and nail bed and can lead to distal phalangeal destruction as the disease progresses. ACH is an incurable disease that requires long-term maintenance therapy to prevent any complications. Because ACH is a variant of pustular psoriasis, it is commonly treated with anti-psoriatic therapies. Unfortunately, it is resistant to many available anti-psoriatic therapies, and there are no clinical guidelines for it; the treatment is therefore highly challenging. Current treatment strategies are mostly based on a few case reports and case series. In this study, we present a case of ACH in a 24-year-old man with a long history of severe skin lesions and overt onychodystrophy, successfully treated with Ustekinumab. In this patient, we observed rapid improvement in skin lesions and symptoms. Ustekinumab can considerably improve other symptoms besides plaque psoriasis. The treatment and excellent outcomes of Ustekinumab may provide clinical benefits to more patients and serve as a reference for other dermatologists.