Harlequin fetus is rare and is the most severe form of congenital ichthyosis, inherited as autosomal recessive trait with an incidence of about 1 in 300,000 births. It is characterized by hyperkeratosis and desquamation of the epidermis which begins prenatally. The skin barrier is severely compromised, leading to excessive water loss, electrolyte imbalance, temperature dysregulation and an increased risk of life threatening infection. In our institution, we report a case of a pregnant woman with a previously born child affected with Harlequin Ichthyosis. For this second pregnancy, they were expecting to have a normal baby since the congenital sonographic scan done prenatally was unremarkable. However, the mother delivered at 29-30 weeks age of gestation with the fetus showing signs of harlequin ichthyosis. The child was a 1200 gram female neonate born prematurely by partial breech extraction. Clinical manifestations of harlequin ichthyosis were present at birth. Furthermore, the fetus suffered from respiratory distress few hours after delivery and died few days after birth. Prenatal diagnosis of HI is made with the use of ultrasound guided fetal skin biopsy, imaging technique with 20 and 30 real time sonography as well as genetic mutational analysis. Prenatal genetic counseling is very essential in her case because of the serious implications to consider for her offspring. The complex management of our patient can be best achieved by a multidisciplinary approach characterized by strong communication, both among the medical team and with the family.