Introduction. Spigelian hernia (SH) or hernia of the Linea semilunaris is an extremely rare disease in the pediatric practice. Though it is a congenital malformation, it is revealed and treated surgically mostly at the age of over 50.Clinical observation. A 6-month-old girl was surgically treated for SH at the Ilyinskaya Hospital. The child had been followed up since her birth for terminal hydronephrosis, complicated by the rupture of renal pelvis. At the age of 4 months, because of absent functions and recurrent infection in the urinary system, the child survived nephroureterectomy. On day 4 after surgery, the patient demonstrated signs of anxiety and pain. Ultrasound examination revealed a strangulated right-sided SH. The hernia was invaginated under ultrasound control. There were no SH relapses during the dynamic follow-up. At the age of 6 months, SH laparoscopic plasty with local tissues was made. There were no complications. There were no any signs of SH relapse for the following 3 months.Conclusions. The search in the literature sources showed that for over 90-year period only 95 cases of SH in children have been described. The article also describes SH direct and indirect signs, possible complications and options for surgical treatment. Laparoscopic SH correction was performed only in 4 cases in children aged 11 months and older. The case presented by the authors is the 5th SH laparoscopic correction in the world.