Abstract. Multifocal granulomatous encephalitis associated with invasion of the brain by Encephalitozoon cuniculi was present in a squirrel monkey less than 24 h old. This appeared to be a congenital infection. The infant's dam was clinically normal.Encephalitozoon (Nosema) cuniculi, a protozoan in the order Microsporidium, is a common parasite of laboratory rabbits, rats, and mice. The organisms characteristically induce granulomatous and nonsuppurative inflammation both in the central nervous system and nonneural tissues. They may also be present without accompanying host response [3]. Usually, regardless of microscopic lesions, the infection is latent or subclinical [4].Encephalitozoonosis has been reported in other species such as dogs, cats, guinea pigs, and hamsters, and has recently been reviewed by SHADDUCK and PAKES [9]. The protozoan is not known to be common in primates. There have been only four reports to date: (1) nonfatal encephalitis in a 9-year-old boy [5] ; (2) fatal, congenitally acquired encephalomyelitis and chorioretinitis in a 4-week-old female human infant (morphology of organisms on light microscopy and certain staining properties were similar to those of Encephalitozoon) [I 11; (3) an asymptomatic infection of intestinal mucosal cells in a Callicebus moloch [8] ; and (4) granulomatous encephalitis, nephritis, and hepatitis, thought by the authors to be congenital [l], in a 2-month-old squirrel monkey.This report is of the second case of encephalitozoonosis in a squirrel monkey, a congenital infection in a perinate.