A 6-year-old girl is referred for an appointment at the University Clinic for Dermatology in Skopje due to her laterally deviated, thickened, yellowish-brown discolored toenails with grooves. The toenail changes began at the age of one, followed by two episodes of nail plate detachment and regrowth of identical toenail plates. Based on a suspected fungal infection, the patient underwent treatment with oral and topical antifungals. Despite the antifungal treatment, no signs of improvement were observed. Based on the clinical presentation, five differential diagnoses came into consideration: physiological nail alterations, pachyonychia congenita, yellow nail syndrome, retronychia, and congenital malalignment of the great toenails (CMGT). Out of the established differential diagnoses, the CMGT matches at most with the clinical presentation of our patient. As in Samman’s description toenails appear yellowish-brown, discolored, oyster-shell-like shaped with a lateral deviation from mild (<100), moderate (10–200), to severe (>200). This lateral deviation can further lead to onychogryphosis, onychomadesis, onychomycosis, retronychia, paronychia, or onycholysis which happened twice to our patient. Most cases of CMGT remain overlooked or are misdiagnosed in up to 85% as onychomycosis. In some patients, CMGT can resolve by itself, while in those with severe deviation, a surgical avulsion can be proposed.