A rare case of syringomyelia associated with Arnold-Chiari malformation, primary 
IgA deficiency and sex chromosomal abnormality is reported. A 26-year-old 
Ethiopian black male was admitted with a complaint of hypalagesia of his left 
arm and face for 10 years. Neurological examination on admission revealed 
dissociated sensory loss of his left arm and face. Mild motor weakness of his 
hand and rotatory nystagmus on left gaze were also noticed. Plain craniogram of 
lateral view showed small posterior cranial fossa with low positioned inion and 
platybasia. MRI with T1-weighted images in sagittal plane revealed tonsillar 
herniation reaching C1 and syrinx extending from C2 to lumbar region. Although 
no episode of infectious disease nor allergy were experienced, blood analysis 
disclosed low serum level of IgA (7 mg/dl). The values of other immunoglobulins 
were within normal range. IgA in saliva was not detected, too. According to the 
clinical history and symptoms, a diagnosis is of primary asymptomatic IgA 
deficiency was obtained. Karyotype analysis showed inversion of Y chromosome. In 
an attempt to avoid anaphylactic shock on blood transfusion in a patient with 
IgA deficiency, autologous blood was prepared before surgery. Decompressive 
craniectomy of the posterior fossa with posterior arch of C1 and C2 was 
performed together with syringosubarachnoid shunt at Th 6-7 level. Postoperative 
course was successful and slight improvement of sensory disturbance was 
obtained. No respiratory or wound infection was occurred. The association of 
these three anomalies is very rare and genetical relationship is not known. From 
surgical point of view, it is conceivable that preoperative management in a case 
of asymptomatic IgA deficiency is uneventful.