Mercury (Hg), and the organometallic compounds formed from it, are among the most toxic of substances to the global environment. Mercury is environmentally ubiquitous, and both wildlife and humans are exposed to the toxic effects of its environmental residues, primarily elemental mercury (Hg0), divalent mercury (Hg2+) and methylmercury (MeHg). Humans are exposed to different forms of Hg, and potential health risks have been reported from such exposures; examples of Hg exposure include mercury vapor from dental amalgams, occupational exposures and exposures during artisan and small-scale gold mining operations. Despite the significance of those foregoing Hg exposures, of particular concern is human and wildlife exposure to MeHg, a potent neurotoxicant. Once incorporated into the body, MeHg easily penetrates the blood-brain barrier and causes damage to the central nervous system, particularly in fetuses. It bioaacumulates and biomagnifies in the aquatic food chain; consequently, fish and seafood consumption is the major pathway by which humans are exposed to MeHg. MeHg is the focus of this review. It adversely affects humans and is currently the subject of intense public health interest and worldwide concern. In this review, I summarize the sources and cycling of global mercury in the environment, pathways of exposure, toxicity and exposure evaluation, toxicokinetics, the common biomarkers to evaluate exposure and effects in populations, and finally review the nutritional risks and benefits from fish consumption.