Traveling north–south through Western North Carolina, it connects the cities and towns of Grover, Shelby, Marion, Spruce Pine and Bakersville.

The byway, which stretches 33 miles (53 km) along NC 226 to Marion, is designated for its extensive views of the South Mountains.

[3][4] Soon after crossing under I-40 (exit 86), NC 226 begins its concurrency with US 221 bypassing west of downtown Marion on a four lane divided freeway.

At the US 70 intersection, travelers can follow highway signs to Mount Mitchell, the highest peak east of the Mississippi River.

After a short concurrency with US 19E, it continues northwesterly towards Bakersville, passing by and through the communities of Minpro, Penland, Normanville and Ledger.

At Iron Mountain Gap (3,727 ft (1,136 m)), NC 226 crosses the Appalachian Trail as it enters Tennessee and continues as SR 107 towards Erwin.

It traversed from the South Carolina state line, near Pineville, north along Park Road into downtown Charlotte.

In 1956, NC 26 was moved onto new road near Dysartville, removing a concurrency with US 64 and establishing one of the earliest grade-separated intersections in the state.