A decreased risk of developing ocular toxoplasmosis (OR = 0.52; CI = 0.32–0.84; P = 0.009; Pc = 0.02) and recurrent manifestations of the disease (OR = 0.13; CI = 0.03–0.45; P = 0.0003; Pc = 0.0009) was observed for the KIR3DS1−/KIR3DL1+/Bw4–80Ile+ combination (KIR2DL1 and the Bw4-80Ile ligand in the absence of KIR3DS1) when compared to patients without ocular toxoplasmosis. Here, KIR2DL1 is linked to ocular toxoplasmosis.