Globozoospermia is a rare (incidence <0.1%) and very severe disorder, with major implications in male fertility. Total globozoospermia is represented by the presence of spermatozoa with 100% rounded heads and a lack of acrosomes. These specific morphological modifications seem to be connected to defects occurring in the last stage of spermatogenesis, spermiogenesis, and will result in anomalies of the acrosomal reaction and a defective adherence of the spermatozoa to the oocytes zona pellucida. This will result in a failure of natural fertilization. This article aims to present the case of a couple diagnosed and successfully treated for primary male infertility. The 26-year-old male partner underwent two semen analyses that revealed the presence of fully rounded spermatozoa heads (morphological abnormality) and consequently was proposed for in vitro fertilization treatment. Semen preparation and the use of assisted reproductive techniques, intracytoplasmic injection of sperm cells into the assisted oocyte activation, have resulted in the conceivement of a healthy child. The particularities of this case lie in the early recognition of the total abnormal globozoospermia morphology. This is the first case reported in Romania where specific assisted reproductive techniques and treatments have resulted in a successful pregnancy for a couple with male total globozoospermia.