The morphological and functional integrity of the acrosome in human spermatozoa is a prerequisite for attachment and binding to the ovum investments in order to allow gamete fusion. Globozoospermia is a rare condition of unknown mode of inheritance which is characterized by the complete absence of the acrosome, disorganized mid-piece, lack of zona binding and infertility. Semen analysis is normal with respect to concentration and motility. Morphological evaluation is necessary for true diagnosis of these patients. Since it is a rare disorder, consultation with andrology clinic is important in diagnostic approach. Two types of globozoospermia were described. Type I is characterized by a spherical arrangement of the chromatin and a complete lack of acrosome, therefore these spermatozoa are unable to penetrate the zona-pellucida, causing primary infertility. Type II has some acrosomal covering with a conical nucleus, which may be surrounded by large droplets of cytoplasmic material indicating secondary degenerative changes. Globozoospermia is believed to have a genetic inheritance, but the exact mode of inheritance has not yet been proven. Autosomal recessive, monogenic and polygenic inheritances were suggested to have a possible role for this unique disease. Many different investigators studied aneuploidy, Y chromosome microdeletion and structural abnormalities of chromatin, but none of them has reported definite mode of inheritance. Before IVF, these patients were completely infertile. Especially after the introduction of ICSI some authors have been reported successful pregnancies and deliveries. But the fertilization rates were lower than other patients, and in some trials pregnancies were obtained only after oocyte activation procedures.