The El Niño Southern Oscillation (ENSO) is a complex climate phenomenon occurring in the Pacific Ocean at intervals of 2–7 years. The term refers to fluctuations in ocean temperatures in the tropical eastern Pacific Ocean (El Niño [the warm phase of ENSO] and La Niña [the cool phase of ENSO]) and in atmospheric pressure across the Pacific basin (Southern Oscillation). This weather pattern is attributed with causing climate change in certain parts of the world and is associated with disease outbreaks. The question of how ENSO affects skin and skin-related disease is relatively unanswered. We aimed to review the literature describing the effects of this complex weather pattern on skin. El Niño has been associated with increases in the occurrence of actinic keratosis, tinea, pityriasis versicolor, miliaria, folliculitis, rosacea, dermatitis by Paederus irritans and Paederus sabaeus, and certain vector-borne and waterborne diseases, such as dengue fever, leishmaniasis, Chagas' disease, Barmah Forest virus, and leptospirosis, and with decreases in the occurrence of dermatitis, scabies, psoriasis, and papular urticaria. La Niña has been associated with increases in the occurrence of varicella, hand, foot, and mouth disease, and Ross River virus (in certain areas), and decreases in viral warts and leishmaniasis. Reports on the effects of ENSO on skin and skin-related disease are limited, and more studies could be helpful in the future.