A 3 year and 3 months old boy with recurrent infections since his age of 5 months was presented with clinical data and autopsy findings. He was the 4th product of healthy parents. His elder brother died of recurrent perianal abscess and sepsis at his age of 3 years. His 2nd elder sister died on the 14th day of life probably from the complication of BCG vaccination. Beginnig with perianal abscesses at his age of 5 months, he has been continuously suffering from recurrent infections such as arthritis, ostomyelitis, pneumonia, epididymitis, subcutaneous abscesses and perianal abscesses. In spite of meticulous supportive and aggressive antibiotic therapy persistent positive cultures for staph. Aureus, klebsiella, E. Coli, Enterococcus and coliform bacilli from different sited were noted. Erythrocyte sedimentation rate of 25 to 40 were constant. White cell count varied frem 15500 to 33400 with polymorphonucleocytes predominance. NBT test showed persistent low scoring of 2% throught the course. He finally died of pneumonia and empyema. At postmortem examination, multiple abscesses and grnulomas of right lung and multipe granulomas in the liver, spleen, lymph node, bone, marrow, adrenal gland, kidney and intestinal wass were noted. At microscopic examination histiocytic granulomas with lipid containing histiocyte infiltrations were noted in every organs described including brain.